United States Department of Agriculture Agricultural Research Service National Agricultural Library Animal Welfare Information Center

Bovine Spongiform Encephalopathy (BSE) and Other Animal Related Transmissible Spongiform Encephalopathies March 2001 (Revised January 2004) AWIC Series #2001-01 Updates Bovine Spongiform Encephalopathy, 1990 Jean Larson United States Department of Agriculture Agricultural Research Service National Agricultural Library Animal Welfare Information Center 10301 Baltimore Avenue Beltsville, MD 20705-2351 Contact us: http://www.nal.usda.gov/awic/contact.php Policies and Links Information on how to request materials that are included in the collection of the National Agricultural Library (NAL) may be found on the Collection Services website at http://www.nal.usda.gov/services/request.shtml. Please read carefully as there are certain restrictions on media and document types.

BSE, also called bovine spongiform encephalopathy or "mad cow disease" is a progressive neurological disorder of cattle that results from infection by an unconventional and extraordinary transmissible agent. BSE is one of several known animal transmissible spongiform encephalopathies including transmissible mink encephalopathy, scrapie, chronic wasting disease of mule deer, and elk, and feline spongiform encephalopathy. The disease name refers to the fact that at the end of the disease, the brain is full of holes like a sponge. The disease may develop in a relatively short time or, as is more usual, will take decades to develop. Scrapie may be the most well known of the spongiform encephalopathies. It occurs in sheep and goats. In general, as in cattle, diseased animals lose coordination of their legs and body movements and eventually cannot stand. The name "scrapie" refers to the fact that the animals can become irritable and develop an intense itch. The unrelenting itch leads them to "scrape" off their wool/fur. Although the majority of the information in this resource is on BSE, the publication covers the recent information on all these diseases as they may affect human health, farm animals and wildlife species.

There are also human conditions that are similar to the animal diseases. In most cases the human diseases are not due to transmissible agents. They can be genetic diseases that run in families, a mutation that happens sporadically in individuals and probably animals as well, or they may be transmitted by ingestion of the infectious agent (e.g. kuru of the Fore people was caused by ritualized cannibalism).

There is still some controversy regarding the nature of the transmissible agent that causes these fatal conditions, but the most accepted theory is that the agent is a modified form of a normal cell surface component known as a prion (proteinaceous infectious articles and (pronounced preeon) protein) (PrP). This modified version of PrP is disease causing, and is both less soluble and more resistant to enzyme degradation then the normal protein. "Currently there is no known treatment for prion diseases, and the fear that prions passed from cattle to humans may be justified." ¹

Dr. Prusiner also states that "prions appear to multiply in an incredible way: they convert normal protein molecules into dangerous ones (PrPsc) simply by inducing the benign molecules to change their shape." "There are hints that the prions causing the diseases " such as BSE and scrapie "may not be the only ones. Prions made of different proteins may contribute to other neurogenerative diseases that are quite prevalent in humans."

It is the transmissible possibilities of the infectious agent moving between animal species and between animals and humans via an oral route that is currently of greatest concern. This concern is due to an outbreak of the BSE disease in United Kingdom (UK) cattle, and the increase of a human spongiform encephalopathy Creutzfeldt-Jakob Disease in the British population that was exposed to meat from cattle that had BSE.

BSE was first recognized in Great Britain in November 1986. The first cases probably occurred in early 1985. It is not definitive that the disease originated from scrapie infected meat and bone meal that was used as a protein supplement in cattle feeds, but there is strong evidence and general agreement that the outbreak was amplified by feeding rendered infected cattle meat-and-bone meal to young calves. "Some other captive ungulates, captive exotic cats and domesticated cats in the UK contracted the disease probably by eating the same feed material. During the peak of the disease (1992), about 1% of the adult cattle in the UK had the disease. Lower incidences have occurred in indigenous cattle in Ireland, France, Switzerland, the Netherlands, and Portugal. A few cases have been recorded in Canada, Germany, Denmark, Italy, the Falkland Islands, and the Sultanate of Oman in animals exported from Great Britain." ²

As of November 2000, in more than 35,000 herds, about 177,500 cases of BSE were confirmed in the UK alone. For current information, see the website of the Office of International Des Epizooties at http://www.oie.int/eng/info/en_esb.htm. According to the Animal and Plant Health Inspection Service of the U.S. Department of Agriculture, the first case of BSE in the United States was confirmed on December 23, 2003. The USDA's web site provides current news and surveillance information on BSE in the United States.

Concurrent with the cattle epidemic in the UK has been a rise of a new variant of CJD (NvCJD) in humans. This form of CJD "predominately affects younger individuals (median age at death 27.5 years as of October 2000), has atypical clinical features, coordination problems within weeks or months, dementia and myoclonus late in the illness, a duration of illness of at least 6 months, and an abnormal brain scan. There is beginning to be strong epidemiologic and laboratory evidence for a causal relationship between the NvCJD and BSE. The absence of confirmed cases of NvCJD in other geographic areas free of BSE supports a causal relationship." ³

Because of the BSE and NvCJD incidence, the Animal Welfare Information Center decided to provide a science based information resource about these diseases and all other potentially important TSEs. This resource includes a bibliographic listing of articles and website resources about the disease, the emergence of the disease in the UK and the political and social events surrounding the BSE crisis. It is a dynamic resource and additional materials on BSE and other TSEs will be added as they are identified.

References:

1. Prusiner, S.B. Prion biology and diseases fatal conformations of proteins during a journey from heresy to orthodoxy. In Prions and Brain Diseases in Animals and Humans. Edited by D.R.O. Morrison. Plenum Press, NY 1998, p. 135-139. 30 refs. ISBN 0-306-45825-X. Part of the NATO ASI series. Series A, Life Sciences: v. 295. It is the proceeding of a NATO Advanced Research workshop on Prions and Brain Diseases in Animals and Humans, held August 19-23, 1996, in Erice, Italy.

2. The Merck Veterinary Manual 8th Edition. eds. S.E. Aiello and A. Mays. Published by Merck & Co., Inc. of Whitehouse Station, NJ. and in cooperation with Merial Limited. Printed by National Publishing Inc. of Philadelphia, PA 1998, p. 897. ISBN: 0-911910-29-8

3. Center for Disease Control, National Center for Infectious Diseases. Questions and Answers Regarding Bovine Spongiform Encephalopathy (BSE) and Creutzfeldt-Jakob Disease. Bovine Spongiform Encephalopathy and Creutzfeldt-Jakob Disease. April, 2001 http://www.cdc.gov/ncidod/dvrd/vcjd/qa.htm

2004

1. Garcia, Rebeca; Jukes, David. The Spanish system of food controls: Its administration and enforcement. Food Control. January 2004; 15(1): 51-59. ISSN: 0956-7135
   NAL call no.: TP372.7.F66
   Descriptors: food safety, BSE, dioxin, European Food Safety Authority (EFSA), EU, Spanish Agency of Food Safety, general structure, Spanish ministries, Spain.

2. Liechti, R. The international conference on bovine spongiform encephalopathy and food safety, April 17-18, 2002. Food Control. January 2004; 15(1): 71-77. ISSN: 0956-7135
   NAL call no.: TP372.7.F66
   Descriptors: current scientific knowledge, BSE, TSEs, food safety risks, a forum for discussion, prion diseases, social science, consumer groups.

3. Moya, K.L.; Hassig, R.; Creminon, C.; Laffont, Di Giamberardino, L. Enhance and retrograde axonal transport of Prpc in peripheral nerve. Journal of Neurochemistry. 2004; 88(1): 155-160. ISSN: 0022-3042.
   NAL call no.: QP351.J6
   Descriptors: PrPC axonal transport, abundance of PrPC in peripheral nerves, prion protein resistant to detergent extraction, prion neuroinvastion, nerve extraction conditions, prion disease application, TSE entry pathway, prion neuroinvasion, scrapie, CNS access.

2003

1. Adjou, Karim Tarik; Simoneau, Steve; Sales, Nicole; Lamoury, Francois; Dormont, Dominique; Papy-Garcia, Dulce; Barritault, Denis; Deslys, Jean Philippe; Lasmezas, Corinne Ida. A novel generation of heparan sulfate mimetics for the treatment of prion diseases.  Journal of General Virology. September 2003; 84(9): 2595-2603.  ISSN:  0022-1317
   NAL call no.:  QR360.A1J6
   Descriptors: transmission of spongiform encephalopathies, PrPres, protease resistant abnormal form, cellular prion protein, heparan sulfate mimetics, HM 2602, effect is to abolish prion propagation in scrapie-infected GT1 cells, in vivo testing, 263K scrapie-infected hamsters, toxicity, dextran sulfate 500, mode of action. 

2. Alexandru, N. Detectia PrPSC in trunchiul cerebral la doua ovine in Romania.  [Detection of PrPSC in the brainstem of two ewes in Romania.]  Revista Romana de Medicina Veterinara. 2003; 13(1): 75-81.  ISSN:  1220-3173. In Romanian with an English summary. 
   NAL call no.:  SF604.R48
   Descriptors: ewes, sheep, natural scrapie diagnosed, prion diseases, brain stem samples by foramen magnum rapid method, immunohistochemistry, Romania. 

3. Belay, Ermias D.; Maddox, Ryan A.; Gambetti, Pierluigi; Schonberger, Lawrence B. Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States.  Neurology. January 28, 2003; 60(2): 176-181. ISSN:  0028-3878
   Descriptors: BSE, 19 European countries, Israel, Japan, vCJD in four European countries, U.S. Centers for Disease Control, National Prion Disease Pathology Surveillance Center, human cases.

4. Bhakdi, S.; Bohl, J. Prions and mad cow disease. Kraftfutter. 2003, 86(3): 56-65. ISSN:  0023-4427.  In English and German.
   NAL call no.:  389.78 K85
   Descriptors: BSE, cattle, cows, bovine spongiform encephalopathy, CJD, epidemiology, prion disease, foodborne meat diseases, new infections declining in UK.

5. Bozzetta, E.; Caramelli, M.; Casalone, C.; Acutis, P.L.; Ru, G. BSE surveillance in Italy: Neuropathological findings in cattle in the frame of the passive surveillance programme.  Journal of Veterinary Medicine, Series A. February 2003; 50(1): 48-49. ISSN: 0931-184X
   NAL call no.:  41.8 Z4
   Descriptors: cattle, BSE, the 272/98/EC Decision, 52 animals with clinical signs, CNS, histopathology, immunohistochemistry (IHC) or Western blot (WB) for PrPSc, no BSE detected, improvement in passive surveillance needed. 

6. Bosques, Carlos J.; Imperiali, Barbara. The interplay of glycosylation and disulfide formation influences fibrillization in a prion protein fragment.  Proceedings of the National Academy of Sciences of the United States of America. June 24, 2003; 100(13): 7593-7598. ISSN:  0027-8424
   NAL call no.:  500 N21P
   Descriptors: prion proteins, PrPC, preteinase K-resistant prion protein scrapie, PrPSc, role of glycosylation, disulfide stability, retarding rate of fibril formation, intermolecular disulfide formation via Cys-179, structure transition. 

7. Brodmann, Peter D.; Moor, Dominik. Sensitive and semi-quantitative TaqManTM real-time polymerase chain reaction systems for the detection of beef (Bos taurus) and the detection of the family Mammalia in food and feed. Meat Science. September 2003; 65(1): 599-607. ISSN: 0309-1740
   NAL call no.:  TX373.M4
   Descriptors: beef, BSE contamination bovine-based products,  vCJD, variant Creutzfeld Jacob Disease, consumer confidence, PCR analysis, mammal DNA, beef DNA, meat and bone meal products, two methods.

8. Brown, David R. Prion protein expression modulates neuronal copper content.  Journal of Neurochemistry. October 2003; 87(2): 377-385.  ISSN:  0022-3042
   NAL call no.:  QP351.J6
   Descriptors: prion protein, copper binding capacity, prion disease, transmissible spongiform encephalopathies, copper content of the brain, transgenic mice animal model, copper levels of the synapse, age effects.

9. Brown, D.A.; Bruce, M.E.; Fraser, J.R. Comparison of the neuropathological characteristics of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) in mice. Neuropathology and Applied Neurobiology. June 2003; 29(3): 262-272.  ISSN: 0305-1846
   Descriptors: neuropathology, inbred mouse strains, RIII, C57BL, VM, C57BL x VM, PrPres deposition, astrocytosis, vacuolation, vCJD linked to BSE, comparison study.    

10. Bruce, Moira E. TSE strain variation. British Medical Bulletin. 2003; 66: 99-108.  ISSN:  0007-1420
    Descriptors: scrapie, cattle, goats, humans, mice, prion disease, strain differences, transmissible spongiform encephalopathy.

11. Concepcion, G. P.; Padlan, E.A. Are humans getting 'mad-cow disease' from eating beef, or something else? Medical Hypotheses.  May 2003; 60(5): 699-701. ISSN:  0306-9877
    Descriptors: hypothesis, gastric digestion of human and various animal prion proteins, ingestion of infected rodent parts, possibly droppings, possible transmission mode of scrapie or BSE to humans.

12. Carp, Richard I.; Kascsak, Richard J. Taking aim at the diagnosis of TSE infectious agents.  Abstracts of Papers American Chemical Society. 2003; 226(1-2): ANYL 9.  ISSN:  0065-7727.  Note:  The 226th American Chemical Society National Meeting, New York, NY, USA, September 07-11, 2003
    NAL call no.:  381 Am33Pa
    Descriptors: BSE, bovine spongiform encephalopathy, diagnosis, prion-disease, transmissible spongiform encephalopathy, variant Creutzfeldt-Jakob disease, vCJD.  

13. Castilla, J.; Gutierrez, Adan A.; Brun, A.; Pintado, B.; Ramirez, M.A.; Parra, B.; Doyle, D.; Rogers, M.; Salguero, F.J.; Sanchez, C.; Sanchez-Vizcaino, J.M.; Torres, J.M. Early detection of PrPres in BSE-infected bovine PrP transgenic mice.  Archives of Virology.  April 2003; 148(4): 677-691. ISSN:  0304-8608
    NAL call no.:  448.3 AR23
    Descriptors: BSE, scrapie, transgenic mouse lines, bovine protein gene expression at different levels, lines exhibited characteristics of bovine disease, PrPres detected, Western blot, immunohistochemistry assays, prions levels of inoculum, pathognomonic markers of disease, incubation period, prion changes from original infection. 

14. Chaala, A.; Roy, C. Recycling of meat and bone meal animal feed by vacuum pyrolysis.  Environmental Science and Technology. October 1, 2003; 37(19): 4517-4522.  ISSN:  0013-936X
    NAL call no.:  TD420 A1E5
    Descriptors: BSE, European beef, alternative disposal of waste animal products and bone meal, vacuum pyrolysis, laboratory reactor, animal flour, combustible gas, high calorific value oil, mineral residue, aqueous phase of organic, pollution control/reduction.

15. Caughey, Byron. Prion protein conversions: Insight into mechanisms, TSE transmission barriers and strains. British Medical Bulletin. 2003; 66: 109-120.  ISSN: 0007-1420
    Descriptors: prions, transmissible spongiform encephalopathies, disease transmission factors, prion protein isoforms, biochemistry, scrapie.

16. Chesebro, Bruce. Introduction to the transmissible spongiform encephalopathies or prion diseases.  British Medical Bulletin.  2003; 66: 1-20.  ISSN:  0007-1420
    Descriptors: TSE, BSE, CJD, prion diseases, etiology, prion protein, disease transmission, theories. 

17. Clauss, M. Do cows fed BSE-infected meat and bone meal in the colostrum-producing stage pass on infectious BSE agent to their calves?  Medical Hypotheses. October 2003; 61(4): 439-443. ISSN:  0306-9877
    Descriptors: BSE, cows, calves, disease transmission, digestion of infectious agent in meat and bone meal, colostrum infectivity, hypothesis.  

18. Cranmer, Morris; McChesney, Thomas. Chronic wasting disease: Risks to hunters and consumers of deer and elk meat.  Neurotoxicology.  March 2003; 24(2): 313-314. ISSN:  0161-813X.  Note: Twentieth International Neurotoxicology Conference:  Emerging Issues in Neurotoxicology, Little Rock, AR, USA, November 18-21, 2002.
    NAL call no.:  RC321.N437
    Descriptors: elk, deer, chronic wasting disease, human health risks, prion disease, transmissible spongiform encephalopathy, meat product, abstract.

19. Dahlanuddin; Van, Tien Dam; Liang, J.B.; Adams, D.B. An exploration of risk factors for bovine spongiform encephalopathy in ruminant production systems in the tropics.  Revue Scientifique et Technique Office International des Epizooties.  April 2003; 22(1): 271-281. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: world risk of BSE, cattle, ruminant production systems, presence of infective agent, transmission and amplification of disease, lack of cattle-based meat and bone meal reduces risks, South East Asia.    

20. Dahms, S. Epidemiologische Modellbildung am Beispiel BSE -- Betrachtungen aus statistischer Sicht. [Epidemiological modelling taking BSE as an example -- reflections from a statistical viewpoint.  Berliner und Munchener Tierarztliche Wochenschrift. 2003, 116(1-2): 22-30.  ISSN: 0005-9366.  In German.
    NAL call no.:  41.8 B45
    Descriptors: BSE, epidemiology modeling, analytical methods, epidemiological surveys, epidemiology, mathematical models, nervous system diseases.  

21. Daude, Nathalie; Marella, Mathieu; Chabry, Joelle. Specific inhibition of pathological prion protein accumulation by small interfering RNAs.  Journal of Cell Science. July 1, 2003; 116(13): 2775-2779. ISSN:  0021-9533
    NAL call no.:  QH301.J6
    Descriptors: transmissible spongiform encephalopathies (TSEs) pathogenesis, PrP, prion protein, proteinase-K resistant isoform, PrPres, small interfering RNA (siRNA) duplexestrigger specific Prnp gene silencing, scrapie-infected neuroblastoma cells, possible therapeutic approach for treatment of prion disease.

22. De Vlieger, J.J.; Puister-Jansen, L.F.; Sengers, H.H.W.J.M.; Ouweltjes, W. Ontwikkelingen in de export van Nederlands fokvee. [Developments in the export of Dutch breeding cattle.]  Rapport, Landbouw Economisch Instituut LEI. 2003; No. 2.03.07, 60 pp.  ISBN:  90-5242-803-4.  In Dutch.
    Descriptors: dairy cattle, Holstein-Friesian, effects of BSE and FMD on markets, animal welfare, European Union, exports, international trade, laws, legislation, market competition, prion diseases, transport of animals, viral diseases, Algeria, Denmark, France, Germany, Lebanon, Netherlands, Poland, Spain.

23. Dedet, V. En tysk ante mortem BSE blodprove vurderes: Forelobige resultater praesenteret pa World Buiatric 2002.  [A German ante-mortem BSE blood test is evaluated. Provisional results presented at the World Buiatric conference, 2002.]  Dansk Veterinaertidsskrift. 2003; 86(2): 24.  ISSN:  0106-6854.  In Danish.
    NAL call no.:  41.9 D23
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, ante-mortem blood test, diagnostic technique, short-strand RNA coated with phospholipids, possible specificity to BSE.  

24. Den Hartog, Johan. Feed for food: HACCP in the animal feed industry.  Food Control. March 2003; 14(2): 95-99.  ISSN: 0956-7135
    NAL call no.:  TP372.7.F66
    Descriptors: Dutch animal feed industry, food and feed safety, GMP standard Animal Feed, BSE and dioxin, integration of HACCP, quality system, proactive approach, the Netherlands.

25. Dimcheff, Derek E.; Portis, John L.; Caughey, Byron. Prion proteins meet protein quality control.  Trends in Cell Biology. July 2003; 13(7): 337-340.  ISSN: 0962-8924
    NAL call no.:  QH573.T73
    Descriptors: protein quality control mechanisms, prion protein aggregation, pathogenesis, proteasome inhibition, neurogeneration.

26. Ducrot, Christian; Roy, Pascal; Morignat, Eric; Baron, Thierry; Calavas, Didier. How the surveillance system may bias the results of analytical epidemiological studies on BSE: Prevalence among dairy versus beef suckler cattle breeds in France. Veterinary Research Les Ulis. March-April 2003; 34(2): 185-192. ISSN:  0928-4249
    NAL call no.:  SF602.A5
    Descriptors: dairy cattle, beef cattle, BSE, disease levels, reliability of surveillance programs, data analysis, Mandatory Reporting Systems of clinically suspect bovines.

27. Ersdal, Cecilie; Simmons, Marion M.; Goodsir, Caroline; Martin, Stuart; Jeffrey, Martin. Sub-cellular pathology of scrapie: Coated pits are increased in PrP codon 136 alanine homozygous scrapie-affected sheep.  Acta Neuropathologica. July 2003; 106(1): 17-28.  ISSN: 0001-6322
    Descriptors: scrapie, sheep, sub-cellular studies, transmissible spongiform encephalopathies, heads of scrapie-affected sheep and controls were perfusion fixed with mixed aldehydes, obexes immunohistochemically labeled, PrP antibodies, electron microscopy of vagal dorsal motor nucleus, coated pits in infected animals, dystrophic neuritis, variable gliosis, plasmalemma invagination, intemalisation.

28. Ersdal, C.; Ulvund, M.J.; Benestad, S.L.; Tranulis, M.A. Accumulation of pathogenic prion protein (PrPSc) in nervous and lymphoid tissues of sheep with subclinical scrapie.  Veterinary Pathology. 2003; 40(2): 164-174. 
    NAL call no.:  41.8 P27
    Descriptors: Rygia breed sheep, pathogenic prion protein, PrPSc, immunohistochemistry, obex, cerebellum, medial retropharyngeal lymph nodes, off spring of PrPSc examined, ileal Peyer’s patch, distal jejunal lymph node, spleen, pathogenesis, prion diseases, BSE.

29. Ferguson, Neil M.; Donnelly, Christl A. Assessment of the risk posed by bovine spongiform encephalopathy in cattle in Great Britain and the impact of potential changes to current control measures.  Proceedings of the Royal Society Biological Sciences, Series B. 7 August 2003; 270(1524): 1579-1584.  ISSN: 0962-8452
    NAL call no.:  501 L84B
    Descriptors: back-calculation model to analyse data, reported clinical cases of bovine spongiform encephalopathy, BSE, analysis of demographic data, levels of human exposure estimated, discussion of possibilities of deaths from variant Cruetzfeldt-Jakob disease, vCJD. 

30. Fries, R.; Eggers, T.; Hildebrandt, G.; Rauscher, K.; Buda, S.; Budras, K.D. Autonomous nervous system with respect to dressing of cattle carcasses and its probable role in transfer of PrPres molecules.  Journal of Food Protection. 2003; 66(5), 890-895.
    NAL call no.:  44.8 J824
    Descriptors: cattle, carcasses, autonomic nervous system, cranial, cervical, and stellage ganglia, the chain of paravertebral ganglia, bovine spongiform encephalopathy, BSE, esophagus, vagus nerve, risk assessment, removal of possible infective material from the food chain.  

31. Galbraith, D.N. Transmissible spongiform encephalopathies and tissue cell culture. Cytotechnology 2003; 39(2): 117-124.  ISSN:  0920-9069
    NAL call no.:  QH585.C97
    Descriptors: TSE, prion proteins, transmission, risks of using living cells and materials for therapeutic compounds, prion biology and pathobiology, discussion of issues.

32. Geldermann, H.; Preuss, S.; Eckert, J.; Han, Y.; Ollesch, K. Analysis of polymorphic microsatellites within the bovine and ovine prion protein (PRNP) genes.  Animal Genetics. August 2003; 34(4): 283-289.  ISSN:  0268-9146
    NAL call no.:  QP98.A1A5
    Descriptors: bovine prion protein gene, microsatellite sites, sheep prion protein genes, distances between microsatellites, pylogenetic origin of alleles, BSE, scrapie, molecular genetics.

33. Gerweck, G. Ein blick auf status der tieraerzte in der lebensmittelueberwachung und tierseuchenbekaempfung. [The status of veterinarians in food surveillance and zoonosis control.]  Tieraerztliche Umschau.  January 1, 2003; 58(1): 40-43.  ISSN:  0049-3864.  In German.
    NAL call no.:  41.8 T445
    Descriptors: zoonotic disease, food safety, role of veterinarians, prion disease surveillance.

34. Ghani, Azra C.; Ferguson, Neil M.; Donnelly, Christl A.; Anderson, Roy M. Factors determining the pattern of the variant Creutzfeldt-Jakob disease (vCJD) epidemic in the UK.  Proceedings of the Royal Society Biological Sciences, Series B.  April 7, 2003; 270(1516): 689-698. ISSN:  0962-8452
    NAL call no.:  501 L84B
    Descriptors: variant of Creutzfeldt-Jakob disease, vCJD, epidemiology, human mortality prediction for 2 and 5 years, exposure to BSE, age dependent susceptibility, incubation period distribution.

35. Gonzalez, Lorenzo; Martin, Stuart; Jeffrey, Martin. Distinct profiles of PrPd immunoreactivity in the brain of scrapie- and BSE-infected sheep: Implications for differential cell targeting and PrP processing.  Journal of General Virology. May 2003; 84(5): 1339-1350.  ISSN:  0022-1317
    NAL call no.:  QR360.A1J6
    Descriptors: scrapie infected sheep, bovine spongiform encephalopathy infected sheep, PrP antibodies, on the disease causing prion protein, immunohistochemical examination of brains, 20 sheep, 4 different PrP antibodies (P4, 521.7, 505.2, R486), strain source differences, source differentiation, cell tropism, PrP processing, variations in PrPd conformation seem influenced by the cell type supporting infection, modulated by the interaction between the infectious agent and the host.

36. Gravenor, M.B.; Ryder, S.J.; Gubbins, S.; Hunter, N.; Baylis, M.; Kao, R.R. Searching for BSE in sheep: interpreting the results so far. Veterinary Record. 2003; 152(10) 298-299.
    NAL call no.:  41.8 V641
    Descriptors: sheep, screening for BSE, scrapie, confidence limit for BSE is no more that 2%, disease prevalence, UK.

37. Grigoletto, G.; Bagordo, F.; Pongolini, S.; Cantoni, A.; Cabassi, E.; Corradi, A. TSE e test diagnostici: valutazione critica e risvolti pratici nel controllo dell'encefalopatia spongiforme bovina.  [TSE and diagnostic tests: critical evaluation and practical implications for the control of bovine spongiform encephalopathy.]  Obiettivi e Documenti Veterinari. 2003; 24(3) 7-16.  In Italian.
    Descriptors: rapid diagnostic tests, BSE, Western blotting, Elisa, comparison study, use and application in Italy, existing legislation, prion diseases, cattle, disease control, Italy.

38. Gubbins, Simon; Simmons, Marion M.; Sivam, Kumar; Webb, Cerian R.; Hoinville, Linda J. Prevalence of scrapie infection in Great Britain: Interpreting the results of the 1997-1998 abattoir survey.  Proceedings of the Royal Society Biological Sciences Series B.  September 22, 2003; 270(1527): 1919-1924. ISSN:  0962-8452
    NAL call no.:  501 L84B
    Descriptors: prevalence of scrapie, sheep, human health risks, sheep transmissible spongiform encephalopathies, TSEs, slaughter plant survey, prevalence of scrapie infection, GB sheep flock of 0.22% (95% confidence interval: 0.01-0.97%).

39. Haywood, S.; Brown, D.R. Transmissible spongiform encephalopathies.  Veterinary Times. 2003; 33(2) 8-9, 10.  ISSN: 1352-9374
    Descriptors: cattle, man, sheep, TSE, CJD, scrapie, binding proteins, copper, manganese, disease transmission, environmental factors manganese. 

40. Heim, D.; Kihm, U. Risk management of transsmissible spongiform encephalopathies in Europe. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 179-199. ISSN:  0253-1933
    NAL call no.:  SF 781.R4
    Descriptors: BSE, scrapie, sheep, goats, cattle, risk management decisions were inaccurate, active and passive surveillance system, ban on feeding meat-and-bone meal (MBM) to ruminants, brain and spinal column as high risk material, European measures. 

41. Hein, Wayne R.; Griebel, Philip J. A road less travelled: Large animal models in immunological research. Nature Reviews Immunology. January 2003; 3(1): 79-84. ISSN:  1474-1733
    Descriptors: many diseases, discussion of large animals as experimental models, viruses, bacteria, TSE’s.

42. Herrmann, Lynn M.; Cheevers, William P.; Davis, William C.; Knowles, Donald P.; O' Rourke, Katherine I. CD21-positive follicular dendritic cells: A possible source of PrPSc in lymph node macrophages of scrapie-infected sheep.  American Journal of Pathology. April 2003; 162(4): 1075-1081. ISSN:  0002-9440
    NAL call no.:  448.8 AM39
    Descriptors: natural sheep scrapie, lymph node analysis, presence PrPSc and macrophage or FDC markers using dual immunohistochemistry, follicular macrophages contain proteases that process full-length PrPSc to N-terminally truncated PrPSc.

43. Hetz, Claudio; Maundrell, Kinsey; Soto, Claudio. Is loss of function of the prion protein the cause of prion disorders? Trends in Molecular Medicine.  June 2003; 9(6): 237-243. ISSN:  1471-4914
    Descriptors: prion diseases, TSE, transmissible spongiform encephalopathies, mechanism of neurogeneration in spongiform encephalopathies is unknown.  

44. Hetz, C.; Soto, C. Protein misfolding and disease: The case of prion disorders.  CMLS Cellular and Molecular Life Sciences. January 2003; 60(1): 133-143.  ISSN:  1420-682X
    NAL call no:  QH301.C45
    Descriptors: TSE, review article, recent data, link between prion protein misfolding, pathogenesis of prion diseases.

45. Hlasny, J. Nektere poznatky z Britanie o historii vyzkumu deficitu horciku u prezvykavcu a BSE. [Some information from United Kingdom concerning the history about Mg- research in ruminants and BSE.] Vyzkum v Chovu Skotu. 2003; 45(1) 22-31.  ISSN:  0139-7265.  In Czech with an English summary.
    Descriptors: cattle, ruminants, BSE, prion diseases, magnesium, mineral deficiencies, United Kingdom.

46. Houston, E.F.; Gravenor, M.B. Clinical signs in sheep experimentally infected with scrapie and BSE.  Veterinary Record. 2003; 152(11): 333-334.
    NAL call no.:  41.8 V641
    Descriptors: sheep, clinical signs, subcutaneous injection, 2g SSBP/1 (scrapie group), intracerebral inoculation with 0.05 BSE brain homogenate, intravenous 0.2g BSE brain homogenate, or 550ml scrapie infected sheep blood, clinical signs compared.

47. Hunter, Nora. Scrapie and experimental BSE in sheep. British Medical Bulletin. 2003; 66: 171-183.  ISSN:  0007-1420
    Descriptors: sheep, BSE, scrapie, experimental infection, Creutzfeldt-Jacob disease, prion disease.

48. Ironside, James W. The spectrum of safety: Variant Creutzfeldt-Jakob disease in the United Kingdom.  Seminars in Hematology.  July 2003; 40(3, Suppl. 3): 16-22. ISSN:  0037-1963
    NAL call no.:  RC633.A1S44
    Descriptors: vCJD, sheep, cattle, prion diseases, BSE, scrapie, transmission, beef, animal feed, meat products, blood supply safety, bone meal products, immune response, UK.

49. Jeffrey, Martin; Martin, S.; Gonzalez, L. Cell-associated variants of disease-specific prion protein immunolabelling are found in different sources of sheep transmissible spongiform encephalopathy.  Journal of General Virology. April 2003; 84(4): 1033-1045. ISSN:  0022-1317
    NAL call no:  QR360.A1J6
    Descriptors: scrapie, BSE, prion disease, prion proteins, BSE and scrapie, intracellular accumulation patterns disease specific prions, (PrPd) lymphoreticular system (LRS)  in sheep brains clinically affected with scrapie or BSE. BSE-infected PrPARQ/ARQ sheep of different breeds compared with scrapie-infected sheep of different PrP genotypes.

50. Kaneider, Nicole C.; Kaser, Arthur; Dunzendorfer, Stefan; Tilg, Herbert; Wiedermann, Christian J.  Sphingosine kinase-dependent migration of immature dendritic cells in response to neurotoxic prion protein fragment.  Journal of Virology. May 2003; 77(9): 5535-5539. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: TSE, circulating dendritic cells mediate neuroinvasion, prion protein expressed in myeloid dendritic cells, prion protein fragment 106-126, chemo-attractant for monocyte-derived immature dendritic cells, signaling events enzymes downstream of Gq protein, inhibition by sphingosine kinase, suggest trans-activation of sphingosine-1-phosphate-dependent cell motility by priori protein.

51. Kang, Shin Chung; Li, Ruliang; Wang, Chuanping; Pan, Tao; Liu, Tong; Rubenstein, Richard; Barnard, Geoff; Wong, Boon Seng; Sy, Man Sun. Guanidine hydrochloride extraction and detection of prion proteins in mouse and hamster prion diseases by ELISA.  Journal of Pathology. April 2003; 199(4): 534-541. ISSN:  0022-3417
    NAL call no.:  448.8 J82
    Descriptors: testing methods, invitro test for TSE, differential extraction, brain homogenates using guanidine hydrochloride followed by DELFIA (Dissociation Enhanced Lanthanide FluoroImmunoAssay), differentiate disease associated PrP isoforms without proteinase K digestion.

52. Kellar, J.A.; Lees, V.W. Risk management of the transmissible spongiform encephalopathies in North America. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 201-225. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: North American Free Trade Agreement partners, Canada, the United States of America (USA), Mexico, harmonized transmissible spongiform encephalopathy (TSE) risk management strategies, quarantine and internal surveillance, BSE, feed bans, scrapie, chronic wasting disease, transmissible mink encephalopathy, national and sub-national veterinary infrastructures, laboratory networks. 

53. Kim, Jae Il; Kuizon, Salomon; Rubenstein, Richard. Comparison of PrP transcription and translation in two murine myeloma cell lines.  Journal of Neuroimmunology. July 2003; 140(1-2): 137-142. ISSN:  0165-5728
    Descriptors: knockout mice myeloma cell lines, hybridomas, prion protein, MAbs. 

54. Kim, Nam Ho; Kim, Jae Il; Carp, Richard I.; Kim, Yong Sun. Effects of transition metals in the conversion mechanism of prion protein and in the pathogenesis of prion diseases.  Current Medicinal Chemistry, Immunology, Endocrine and Metabolic Agents. June 2003; 3(2): 149-160. ISSN:  1568-0134
    Descriptors: prion protein, cattle, CJD, scrapie, TSE, scrapie, prion diseases, pathogenesis.  

55. Kimura, Nobuhiro. BSE outbreak and feed security.  Japanese Poultry Science.  May 2003; 40(J2): J98-J104.  ISSN:  0029-0254.  In Japanese.
    NAL call no.:  47.8 N57
    Descriptors: food safety and security, public health risks, bovine spongiform encephalopathy, BSE, epidemiology, prion disease, transmission.

56. Klass, Michael R.; Hodges, Steven; Sayers, Riona; Clarke, John; Lyons, Vanessa. Testing for TSE: Mad cows, scrapie sheep and wasted deer and elk.  Abstracts of Papers American Chemical Society. 2003; 226(1-2): ANYL 10. ISSN:  0065-7727.  Note:  226th American Chemical Society National Meeting, New York, NY, USA, September 7-11, 2003
    NAL call no.:  381 AM33Pa
    Descriptors: cattle, deer, elk, sheep, scrapie, BSE, chronic wasting disease, testing, prion protein detection, ELISA, immunologic techniques, laboratory techniques, Enfer-TSE-test, Europe, Ireland, USA.

57. Kocisko, David A.; Baron, Gerald S.; Rubenstein, Richard; Chen, Jiancao; Kuizon, Salomon; Caughey, Byron. New inhibitors of scrapie-associated prion protein formation in a library of 2,000 drugs and natural products.  Journal of Virology.  October 2003; 77(19): 10288-10294. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: prion protein, scrapie-infected mice neurobalstoma cells, scrapie strain RML, high-throughput screening assay for PrPSc, 96 well format, polyphenols (e.g., tannic acid and tea extracts), phenothiazines, antihistamines, statins, antimalarial compounds.

58. Larski, Zdzislaw. Niektore nowe dane dotyczace wirusologii i zakaznych gabczastych encefalopatii. [Some new data concerning virology and transmissible spongiform encephalopathies.]  Medycyna Weterynaryjna. 2003; 59(2): 95-99.  ISSN:  0025-8628.  In Polish.
    NAL call no.:  41.8 M463
    Descriptors: review article, lipid rafts, virons, various diseases, imbalance of trace elements and changes in antioxidant function of prion protein, BSE in sheep vs scrapie, cannibalism as a cause of BSE.

59. Lasmezas, C.I. The transmissible spongiform encephalopathies. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 23-36.  ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: TSE’s, Creutzfeldt Jakob disease, CJD, etiology, prion diseases, transmission, prevention and control, scrapie,  risk management, general features of the diseases, mode of replication, pathogenesis, molecular basis of PrP accumulation.

60. Ledoux, J.M. Features of the comparative pharmacokinetics of lithium; a potential application of its use in livestock farming.  Medical Hypotheses. August 2003; 61(2): 278-281. ISSN:  0306-9877
    Descriptors: cattle, mink, lithium’s neuroprotective and neurotropic properties, treatment for sub-acute transmissible spongiform encephalopathies, proposed pharmacokinetic tests.

61. Leucht, Christoph; Simoneau, Steve; Rey, Clemence; Vana, Karen; Rieger, Roman; Lasmezas, Corinne Ida; Weiss, Stefan. The 37 kDa/67 kDa laminin receptor is required for PrPSc propagation in scrapie-infected neuronal cells. EMBO Reports. March 2003; 4(3): 290-295. ISSN:  1469-221X
    NAL call no.:  QH506.E46
    Descriptors: prions, scrapie infected, neuronal cells, infection prevention, PrPSc, laminin receptor (LRP/LR) is necessary for PrPSc propagation in vitro, LRP/LR-specific antibodies as possible therapeutic tools for transmissible spongiform encephalopathies.

62. Lewicki, Hanna; Tishon, Antoinette; Homann, Dirk; Mazarguil, Honore; Laval, Francoise; Asensio, Valerie C.; Campbell, Iain L.; DeArmond, Stephen; Coon, Bryan; Teng, Chao; Gairin, Jean Edouard; Oldstone, Michael B.A. T cells infiltrate the brain in murine and human transmissible spongiform encephalopathies.  Journal of Virology.  March 2003; 77(6): 3799-3808. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: CD4 and CD8 T lymphocytes infiltrate parenchyma, mouse brains, intracerebral, intraperitoneal, or oral inoculation, Chandler strain of mouse scrapie, pattern compared to prion protein knockout (PrP-/-) mice, MHC class I and II molecules, elevated levels of T-cell chemokines, macrophage inflammatory protein 1beta, IFN-gamma-inducible protein 10, and RANTES, PrPSc in CNS associated with chemokines.

63. Liu, Wing Gee; Brown, Debbie A.; Fraser, Janet R. Immunohistochemical comparison of anti-prion protein (PrP) antibodies in the CNS of mice infected with scrapie.   Journal of Histochemistry and Cytochemistry.  August 2003; 51(8): 1065-1071. ISSN:  0022-1554
    NAL call no.:  381 J822
    Descriptors: transmissible spongiform encephalopathies, TSEs, tissue fixative affects, formol saline, periodate lysine paraformaldehyde,  PLP, MAbs, 6H4, 7A12 and 8H4 revealed targeted PrPsc labeling, scrapie mouse models.

64. Lloyd, T.; McCorriston, S.; Morgan, W.; Chern, W.S. (ed.); Rickertsen, K. How do markets respond to food scares? Health, Nutrition and Food Demand. 2003; 247-270.  ISBN: 0-85199-647-7
    NAL call no.:  381 J8222
    Descriptors: food scares, beef, bovine spongiform encephalopathy, impact on consumer attitudes, consumer behavior, food safety and consumer protection, economics, food consumption, food contamination, food hygiene and safety, food-intake, market economics, humans, UK.  

65. Lucassen, Ralf; Nishina, Koren; Supattapone, Surachai. In vitro amplification of protease-resistant prion protein requires free sulfhydryl groups.  Biochemistry April 15 2003; 42(14): 4127-4135.  ISSN:  0006-2960
    NAL call no.:  381 B523
    Descriptors: PrPSc, prion misfolding, molecular mechanism of misfolding, in-vitro PrPSc amplification techniques, scrapie infected brain homogenate, in vitro amplification with Syrian hamster Sc237PrPSc, pH7 and CD-1 mouse RML PrPSc pH6, thiolate-specific alkylating agent N-ethylmaleimide (NEM), reversible thiol-specific blockers p-hydroxymercuribenzoic acid (PHMB) and mersalyl acid inhibited PrPSc amplification.

66. Mabbott, Neil A.; Young, Janice; McConnell, Irene; Bruce, Moira E. Follicular dendritic cell dedifferentiation by treatment with an inhibitor of the lymphotoxin pathway dramatically reduces scrapie susceptibility.  Journal of Virology. June 2003 2003; 77(12): 6845-6854. ISSN:  0022-538X
    NAL call no.:  QR360.J6
    Descriptors: transmissible spongiform encephalopathies, TSEs, mouse scrapie animal model of disease, dendritic cells, prion protein for replication in lymphoid tissue and subsequest neuroinvasion, lymphotoxin beta receptor-immunoglobulin fusion protein (LTbetaR-Ig), temporary dedifferentiation, intraperitoneal scrapie inoculation blocked early accumulation of PrPSc in spleen, reduced disease susceptibility, 28 and 49 days, routes of exposure, possible early intervention.

67. Mainsant, P. Meat consumption before and after BSE in France and the European Union.  Sciences des Aliments. 2003; 23(1): 37-39.  ISSN:  0240-8813
    NAL call no.:  TX341.S34
    Descriptors:  consumer responses, meat and meat product consumption, bovine spongiform encephalopathies, beef cattle, comparison study, EU.

68. Manuelidis, Laura. Transmissible encephalopathies: Speculations and realities.  Viral Immunology. Summer 2003; 16(2): 123-139. ISSN:  0882-8245
    Descriptors: scrapie, CJD, BSE, prion theory, review article, PrP, transmission factors, pathology, response to infectious agent, possible viral caused disease.

69. Matthews, D.; Cooke, B.C. The potential for transmissible spongiform encephalopathies in non-ruminant livestock and fish.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 283-296. ISSN: 0253-1933
    NAL call no.:  SF781.R4
    Descriptors: pigs, poultry, susceptibility to BSE agent, parenteral challenge causes infection in pigs, oral exposure to BSE-infected cattle brain, oral challenge with sheep scrapie, chickens show resistant to oral challenge with sheep scrapie.

70. Matthews, D. BSE: A global update.  Society for Applied Microbiology Symposium Series. 2003; (32): 120S-125S. ISSN:  1467-4734
    NAL call no.:  QR1.S64
    Descriptors: BSE, active surveillance in Europe, Britain’s epidemic, British feed controls, epidemiology, cattle, European Commission's Scientific Steering Committee considering risks of importing BSE, consumer concerns, dispersal of infectivity from European countries was widespread.

71. McCrea, D. Risk communication of the transmissible spongiform encephalopathies.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 251-257. ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: risk communication, public, prion disease, epidemiology, disease prevention and control, transmission, transmissible spongiform encephalopathy, food products, food safety, risk communication, definitions, goals.

72. McKintosh, Edward; Tabrizi, Sarah J.; Collinge, John. Prion diseases.  Journal of Neurovirology. April 2003; 9(2): 183-193. ISSN:  1355-0284
    Descriptors: BSE, vCJD, history, epidemiology, prion diseases, TSE, current research human prion disorders.

73. Miele, G.; Blanco, A.R. Alejo; Baybutt, H.; Horvat, S.; Manson, J.; Clinton, M. Embryonic activation and developmental expression of the murine prion protein gene. Gene Expression. 2003; 11(1): 1-12. ISSN:  1052-2166
    NAL call no.:  QH450.G46S
    Descriptors: prion protein, PrP mRNA expression, murine embryos and various adult tissues, expression PrP RNA not in adult kidney and liver, investigated effected superoxide radicalin cultured neuroblastoma and astrocyte cells, suggest that PrPC is part of cellular antioxidant defense mechanism.

74. Morley, R.S.; Chen, S.; Rheault, N. Assessment of the risk factors related to bovine spongiform encephalopathy.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 157-178.  ISSN:  0253-1933
    NAL call no.:  SF781.R4
    Descriptors: status of BSE, cattle populations, risk assessment, surveillance criteria, International Animal Health Code, consumption of meat-and–bone-meal (MBM) by cattle, importation of cattle, possible contamination of MBM, livestock population structure, rendering processes, animal feeding practices. Application of the OIE, BSE guidelines, costs and losses with introduction and establishment of BSE in other countries, Canada.   

75. Nishida, Yuzo. Elucidation of endemic neurodegenerative diseases: A commentary. Zeitschrift fuer Naturforschung Section C, Journal of Biosciences. September-October 2003; 58 (9-10): 752-758. ISSN: 0939-5075
    NAL call no.:  QH301.Z4
    Descriptors: scrapie, CJD, chronic wasting disease, soil metal ion levels, copper, manganese, iron, aluminum, cellular accumulation of metallic ions, acid rain effects on solubility, iron-overload syndrome, hydrogen peroxide, prion isoforms, PrPc and PrPSc, Iceland, Slovakia, Colorado.

76. Nishida, Noriyuki; Sakaguchi, Suehiro; Katamine, Shigeru. Prion disease and antiprion substance.  Journal of Pharmacological Sciences. 2003; 91(Supplement I): 48P. ISSN: 1347-8613.  Note:  76th Annual Meeting of the Japanese Pharmacological Society, Fukuoka, Japan, March 24-26, 2003
    Descriptors: anti-prion substance, Congo Red, prion disease, drug screening system, cell culture model, histology and cytology techniques.

77. Nunziante, Max; Gilch, Sabine; Schaetzl, Hermann M. Essential role of the prion protein N terminus in subcellular trafficking and half-life of cellular prion protein.  Journal of Biological Chemistry. February 7, 2003; 278(6): 3726-3734.  ISSN:  0021-9258
    NAL call no.:  381 J824
    Descriptors: prion protein life history, biochemistry, glycosylphosphatidylinositol anchor, prion-protein N-terminus, cellular half-life, transmissible spongiform encephalopathies.

78. O'Rourke, Katherine I.; Knowles, Donald P.; Baszler, Timothy V.; Parish, Steven M.  Methods for detection of prion protein as an indication of transmissible spongiform encephalophathies. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] Feb. 4, 2003; 1267(1): No Pagination.  ISSN:  0098-1133
    NAL call no.:   T223.A21
    Descriptors: PrPSc, prion protein, detection methods, diagnostic techniques. infected live animals, postmortem detection methods, third eyelid-associated lymphoid tissue, monoclonal antibodies, conserved epitope of PrPSc protein, fixed or frozen tissue.

79. Ochel, H. J.; Gademann, G.; Trepel, J.; Neckers, L. Modulation of prion protein structural integrity by geldanamycin.  Glycobiology.  September 2003; 13(9): 655-660.  ISSN:  0959-6658
    NAL call no.:   QP552.G59G593
    Descriptors: prion protein, transmissible spongiform encephalopathies, HSP90 inhibitors, geldanamycin or radicicol effects, eukaryotic cells, tunicamycin, bands of western blot analysis.

80. Office International des Epizooties. Risk analysis of prion diseases in animals. Revue Scientifique et Technique Office International des Epizooties. 2003; 22(1), 344 pp.  ISSN:  0253-1933.  In English, French, and Spanish.
    NAL call no.:   SF781.R4
    Descriptors: BSE, TSE, scrapie, prion diseases, current and established knowledge, risk assessment, management in different countries, diagnosis and disease prevention.    

81. Ozawa, T.; Lopez-Villalobos, N.; Blair, H.T. Beef traceability systems in Japan: How should New Zealand prepare? Proceedings of the New Zealand Society of Animal Production. 2003; 63: 49-52.  ISSN:  0370-2731
    NAL call no.:   49.9 N483
    Descriptors: BSE, tracing systems, farm to consumer systems, not affective in Japan or other countries, question of expenses vs expected financial to NZ beef producers, Ministry of Agriculture, Forestry and Fisheries, National Federation of Agricultural Cooperative Association, New Zealand Meat Board.  

82. Ozawa,Y. Risk management of transmissible spongiform encephalopathies in Asia. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 237-249. ISSN: 0253-1933
    NAL call no.:   SF781.R4
    Descriptors: questionnaire-based survey, distributed to the Office International des Epizooties Member Countries in Asia, risk management for transmissible spongiform encephalopathies, 16 responses, risk analysis not in 10 countries, ruminant origin feed stuff imported into Asia, upgrading of surveillance and notification programs recommended, BSE, scrapie.

83. Peelman, L.J.; van Poucke, M. Een eerste bepaling van de PRNP-genotypenfrequenties bij de voornaamste schapenrassen in Belgie. [The first determination of PRNP genotype frequency in the most important sheep breeds in Belgium.] Vlaams Diergeneeskundig Tijdschrift. 2003; 72(1): 20-26. In Dutch with an English summary.
    NAL call no.:   41.8 V84
    Descriptors: scrapie, sheep, selection of scrapie resistant sheep, ARR allele, sampled most breeds use in Belgium, 854 animals, VrQ/VrQ and ARR/ARR genotype.

84. Pennington, H. Science, governments and microbes: Food safety in the 21st century.  FEMS Congress of European Microbiologists Abstract Book. 2003; (1): 1. Note:  1st Federation of European Microbiological Societies (FEMS) Congress of European Microbiologists, Ljubljana, Slovenia, June 29-July 03, 2003.
    Descriptors: research, governmental policies, infectious organisms, food borne pathogens, bacteria, E. coli O157, production animals, prions, viruses, BSE.

85. Periago, P.M.; Fernandez, A.; Collado, J.; Martinez, A. Note: Use of a distribution of frequencies model to interpret the tailed heat inactivation curves of prions. Food Science and Technology International. February 2003; 9(1): 29-32. ISSN:  1082-0132
    NAL call no.:   TP368.F66S
    Descriptors: BSE, bovine spongiform encephalopathy, prion-disease, Weibull models, two parameter empirical model, mathematical and computer techniques for statistical analysis, canned food industry, kinetics, prion inactivation curves, different temperatures under different conditions, Europe.

86. Polak, M.P.; Zmudzinski, J.F.; Larska, M.; Rozek,W.; Kozaczynski, W.; Reichert, M.  Skutecznosc systemu monitorowania BSE w Polsce na przykladzie pierwszych wykrytych przypadkow choroby. [Efficacy of the BSE monitoring system in Poland [analysis of the first four cases].  Zycie Weterynaryjne. 2003; 78: 2, 97-99.  ISSN:  0137-6810.  In Polish with an English summary.
    NAL call no.:   SF604.Z9
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, case reports, disease monitoring, immunological techniques, public health concerns, Poland.  

87. Prendergast, D.M.; Sheridan, J. J.; Daly, D. J.; McDowell, D.A.; Blair, I.S. Dissemination of central nervous system tissue from the brain and spinal cord of cattle after captive bolt stunning and carcass splitting.  Meat Science. December 2003; 65(4): 1201-1209. ISSN:  0309-1740
    NAL call no.:   TX373.M4
    Descriptors: BSE, cattle, measures to exclude specified risk materials from human food chain, CNS tissue dissemination, slaughter practices, captive bolt stunning, carcass splitting, CNS material contamination of meat.

88. Polak, Miroslaw P.; Larska, Magdalena; Zmudzinski, Jan F. Nowe szybkie testy do diagnostyki post mortem BSE. [New rapid tests for BSE post mortem diagnosis.]  Medycyna Weterynaryjna. 2003; 59(10): 876-878.  ISSN:  0025-8628. In Polish.
    NAL call no.:   41.8 M463
    Descriptors: cattle, BSE, rapid diagnostic test comparison, screening methods and techniques, sensitivity, specificity, limiting detectable levels, PrPSc, prions, Scientific Steering Committee of the European Commission, post-mortem testing.

89. Purdey, Mark. Does an infrasonic acoustic shock wave resonance of the manganese 3+ loaded/copper depleted prion protein initiate the pathogenesis of TSE?  Medical Hypotheses.  June 2003; 60(6): 797-820.  ISSN:  0306-9877
    Descriptors: copper, Cu, Mn3+, hyper-polarization, paramagnetic-status, piezoelectric atomic structure, cupro-protein expression, transmissible spongiform encephalopathy, prion diseases, UV radiation, acoustic radiation, circadian auditory pathway, electromagnetic superexchange, electron phonon coupling, endogenous electromagnetic energy, geomagnetic radiation, infrasound rich environment, tetonic disturbances, supersonic airplanes, ratios of high MN/low copper and low zinc of mammals brains, 2 stage TSE pathogenesis initiated when MN substitutes for vacant Cu domain on PrPC (sleeping) when infrasonic shock the atomic structure of Mn3+ starts the distortion of the protein, Cu prions replaced by hyperpolarized Mn 3+ prions, self perpetuating 'cluster bombs' of free radical mediated neurodegeneration.  

90. Rachidi, Walid; Mange, Alain; Senator, Abderrahmene; Guiraud, Pascale; Riondel, Jacqueline; Benboubetra, Mustapha; Favier, Alain; Lehmann, Sylvain. Prion infection impairs copper binding of cultured cells.  Journal of Biological Chemistry. April 25 2003; 278(17): 14595-14598. ISSN:  0021-9258
    NAL call no.:   381.J824
    Descriptors: TSE, molecular mechanism of neurogeneration, 64CU, radioactive copper, prion infected cells, reduction in copper binding, prion infection modulates copper content at a cellular level, modification of copper homeostasis has determinant role in neuropathology.

91. Richard, Marlene; Biacabe, Anne Gaelle; Streichenberger, Nathalie; Ironside, James West; Mohr, Michel; Kopp, Nicolas; Perret, Liaudet Armand. Immunohistochemical localization of 14.3.3 zeta protein in amyloid plaques in human spongiform encephalopathies.  Acta Neuropathologica. March 2003; 105(3): 296-302.  ISSN: 0001-6322
    Descriptors: MV2 CJD, kuru, VV2cjd, vCJD, Gerstmann-Straussler-Scheinker, Alzheimer, paraffin-embedded brain section, different subtypes of brain amyloid plaques, comparison of PrPSc and 14.3.3 zeta deposits presence, data suggests, 14.3.3 zeta protein could interact with PrP, other components of PrPSc deposites in CJD, immunostaining.

92. Rico, Andre. Prion: Toxic or infectious agent?  Medical Hypotheses.  February 2003; 60(2): 209-214.  ISSN:  0306-9877
    Descriptors: TSE, caused by deficiency of chemo-defense system (CDS), unable to destroy or eliminate PrPsc, immune defense system (IDS) accommodates PrPsc as an inert particle that is moved through lymphoreticular system, PrPSc acts as cellular toxic disruptor of post-translational phase of PrP biosynthesis. 

93. Ridley, Rosalind M. What would T. H. Huxley have made of prion diseases? Molecular Biotechnology. July 2003; 24(3): 243-256. ISSN:  1073-6085
    NAL call no.:   TP248.13.M65
    Descriptors: speculating on T.H. Huxle’s thoughts regarding transmissible spongiform encephalopathies, scientific debate over prion hypotheses.  

94. Roels, Stefan L.M. F.; De Meyer, Gaelle; Tedik, Kamile; Foubert, Raphael; Vanopdenbosch, Emmanuel. Variation of mass (volume) taken with the calibrated syringe and of the results provided by the Bio-Rad PlateliaTM BSE test upon storage of brainstem samples at -20degrees C.  Animal Research. November-December 2002(2003); 51(6): 493-499. ISSN: 1627-3583
    Descriptors: suspected BSE brainstem material, sampling with a kit calibrated syringe, quick PlateliaTM test from Bio-Rad, decrease in optical density after 1 week cold storage, density change did not affect diagnosis of BSE. 

95. Rudyk, Helene; Knaggs, Michael H.; Vasiljevic, Snezana; Hope, James; Birkett, Chris; Gilbert, Ian H. Synthesis and evaluation of analogues of congo red as potential compounds against transmissible spongiform encephalopathies. European Journal of Medicinal Chemistry.  June 2003; 38(6): 567-579.  ISSN:  0223-5234
    Descriptors: anti-TSE compounds, analogues, Congo red 4, 6, 8, diazonium salts,  symmetrical bis azoic dyes 14-19, 21-22, 24 and 26-29 as their sodium salts, molecular modeling, potential structure activity relationships.

96. Salman, Mo D. Chronic wasting disease in deer and elk: Scientific facts and findings.  Journal of Veterinary Medical Science. July 2003; 65(7): 761-768. ISSN:  0916-7250
    NAL call no.:   SF604.J342
    Descriptors: deer, elk, chronic wasting disease, CWD, disease history, pathogenesis, susceptibility of animals, transmission, potential origins, diagnostic methods, control strategies, economic impact, food and food safety, public health risks, infectivity to humans, North America.

97. Schreuder, B.E.C.; Somerville, R.A. Bovine spongiform encephalopathy in sheep?   Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 103-120.  ISSN:  0253-1933 ISSN:  0253-1933
    NAL call no.:   SF781.R4
    Descriptors: BSE infected sheep hypothetical, risk management and pre-emptive measures, experimental BSE in sheep, differentiating BSE and scrapie in same host, classical strain typing in a mouse model.   

98. Sellier, P. Protein nutrition for ruminants in European countries, in the light of animal feeding regulations linked to bovine spongiform encephalopathy.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 259-269.  ISSN:  0253-1933
    NAL call no.:   SF781.R4
    Descriptors: BSE, protein in production animal diets, new sources of protein, minerals and lipids in animal diets, manufactured concentrates, vegetals as feed source alternatives, feed regulations, implementation and management, Europe, World.   

99. Simoneau, Steve; Haik, Stephane; Leucht, Christoph; Dormont, Dominique; Deslys, Jean Philippe; Weiss, Stefan; Lasmezas, Corinne. Different isoforms of the non-integrin laminin receptor are present in mouse brain and bind PrP.  Biological Chemistry.  February 2003; 384(2): 243-246. ISSN:  1431-6730
    NAL call no.:   QP501.B56
    Descriptors: prion protein, mouse brain fractions, laminin receptor, isoforms interaction with prion protein, physiological role, laminin receptor/PrP interaction in the brain, relevance for transmissible spongiform encephalopathies.

100. Smith, Peter G.; Bradley, Ray. Bovine spongiform encephalopathy (BSE) and its epidemiology. British Medical Bulletin. 2003; 66: 185-198.  ISSN:  0007-1420
     Descriptors: BSE, cattle, food safety, sheep, humans, processed animal waste, prion diseases, epidemiology, UK.

101. Smith, Peter G. The epidemics of bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: Current status and future prospects.  Bulletin of the World Health Organization.  2003; 81(2): 123-130.  ISSN:  0042-9686
     NAL call no.:   449.9 W892B
     Descriptors: BSE, epidemic, Europe, UK, control measures, EU, Switzerland, testing post mortem, tests for live animals needed, risks small and diminishing in Europe, 150 diagnosed with vCJD, control of iatrogenic transmission via blood transfusion or contaminated surgical instruments, new tests, decontamination methods needed. 

102. Speare, Jonathan O.; Rush, Thomas S. III; Bloom, Marshall E.; Caughey, Byron. The role of helix 1 aspartates and salt bridges in the stability and conversion of prion protein.  Journal of Biological Chemistry. April 4 2003; 278(14): 12522-12529. ISSN:  0021-9258
     NAL call no.:   381.J824
     Descriptors: pathogenesis,  transmissible spongiform encephalopathies, conversion of PrPsen to PrPres, constructed mutants of hamster prion protein, replacing aspartic acids, salt bridges, cell-free conversion data, Asp-144 and Asp-147 and the respective salt bridges stabilize PrPsen from converting to PrPres. 

103. Stoltze, L.; Rezaei, H.; Jung, G.; Grosclaude, J.; Debey, P.; Schild, H.; Rammensee, H.G. CD4+ T cell-mediated immunity against prion proteins.  CMLS—Cellular and Molecular Life Sciences. March 2003; 60(3): 629-638.  ISSN:  1420-682X
     NAL call no.:   QH301.C45
     Descriptors: prion protein, amino sequences of PrP, differences in amino acid sequence, presentation of distinct peptides on major histo-compatibility complex class II molecules, activation of specific CD4+ T cells, effective immune response against foreign PrP, antibody production, distinguish self and foreign. 

104. Tahiri, Alaoui Abdessamad; Bouchard, Mario; Zurdo, Jesus; James, William.  Competing intrachain interactions regulate the formation of beta-sheet fibrils in bovine PrP peptides.  Protein Science. March 2003; 12(3): 600-608. ISSN: 0961-8368
     NAL call no.:   QD431.A1P78
     Descriptors: bovine prion protein biochemistry, pathogenesis of TSE, PrPC helix converted to aberrant beta-sheet-dominated form (PrPSc), peptide analysis.  Native PrPC helix 1 might inhibit the strong intrinsic beta-sheet-forming propensity of sequences immediately N-terminal to the globular core of PrPC, by keeping in place intrachain interactions that would prevent these amyloidogenic regions from triggering aggregation.

105. Taylor, D. M.; Woodgate, S.L. Rendering practices and inactivation of transmissible spongiform encephalopathy agents. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 297-310. ISSN: 0253-1933
     NAL call no.:   SF781.R4
     Descriptors: BSE, scrapie, meat and bone meal, rendering of infected animal tissues, safety of tallow and tallow by-products, relationship between BSE and variant Creutzfeldt-Jakob disease, vCJD, UK.

106. Terry, L.A.; Marsh, S.; Ryder, S.J.; Hawkins, S.A.C.; Wells, G.A.H.; Spencer, Y.I.  Detection of disease-specific PrP in the distal ileum of cattle exposed orally to the agent of bovine spongiform encephalopathy. Veterinary Record. 2003; 152(13): 387-392.
     NAL call no.:   41.8 V641    
     Descriptors: BSE, cattle, distal ileum, PrPSc, mesemteric lymph nodes, experimental orally dosed animals, naturally occurring clinical cases, Peyer’s patches, differences, immunohistochemistry, macrophages. 

107. Thackray, Alana M.; Madec, Jean Yves; Wong, Edmond; Morgan, Warren Robert; Brown, David R.; Baron, Thierry; Bujdoso, Raymond. Detection of bovine spongiform encephalopathy, ovine scrapie prion-related protein (PrPSc) and normal PrPc by monoclonal antibodies raised to copper-refolded prion protein.  Biochemical Journal.  15 February 2003; 370(1): 81-90.  ISSN:  0264-6021
     NAL call no.:   QP 501.B64
     Descriptors: BSE, scrapie, prion proteins, monoclonal antibodies for copper-refolded PrP, diagnosis of normal and abnormal forms of protein, disease-susceptible allelic form V136R154Q171 ('VRQ'; where single-letter amino-acid notation has been used) and disease-resistant allelic form A136R154R171 ('ARR') of recombinant ovine PrPc, reaction unglycosylated and monoglycosylated forms of PrPSc from prion-infected tissue samples, different species tested by Western blot, distinquished  between bovine spongiform encephalopathy & scrapie PrPSc from experimentally infected sheep due to different electrophoretic mobilities.

108. Thomzig, Achim; Kratzel, Christine; Lenz, Gudrun; Krueger, Dominique; Beekes, Michael. Widespread PrPSc accumulation in muscles of hamsters orally infected with scrapie. EMBO Reports. May 2003; 4 (5): 530-533.  ISSN:  1469-221X
     NAL call no.:   QH506.E46
     Descriptors: hamsters, animal model of disease, scrapie, BSE, chronic wasting disease, TSE, spongiform encephalopathies, prion proteins, PrPSc, western blotting, skeletal muscle levels, forelimb, hindlimb, head, back, shoulder, tongue, peripheral routing of infection, question whether muscles contain the infectious agent.

109. Travis, Dominic; Miller, Michele. A short review of transmissible spongiform encephalopathies, and guidelines for managing risks associated with chronic wasting disease in captive cervids in zoos.  Journal of Zoo and Wildlife Medicine. June 2003; 34 (2): 125-133. ISSN:  1042-7260
     NAL call no.:   SF601.J6
     Descriptors: prion diseases, transmissible spongiform encephalopathies, TSE, scrapie, kuru, variant Creutzfeld-Jakob disease, review article, zoo and wildlife veterinarians, wildlife biologists, chronic wasting disease, cervids, risk management strategies, disease prevention.

110. Trevitt, Clare R.; Singh, Pramil N. Variant Creutzfeldt-Jakob disease: Pathology, epidemiology, and public health implications.  American Journal of Clinical Nutrition. September 2003; 78(3 Supplement): 651S-656S. ISSN:  0002-9165
     NAL call no.:   389.8 J824
     Descriptors: BSE, vCJD, transmissible spongiform encephalopathies, cattle, sheep, scrapie, prion diseases, review, outline public health implications, data, emphasizing preventative measures, areas of research for screening and diagnosis.

111. Van Gelderen, C.; Gimeno, E.J.; Schudel, A.A. Bovine spongiform encegphalopathy in South America: A regional preventive approach.  Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 227-236.  ISSN:  0253-1933
     NAL call no.:   SF781.R4
     Descriptors: bovine spongiform encephalopathy, BSE, cattle, prion diseases, disease prevention, safeguarding animal health, preventative action adopted in 1989, region is free of BSE, South American programs, harmonizing BSE prevention programs, maintenance of disease free status.

112. Watt, Nicole T.; Hooper, Nigel M. The prion protein and neuronal zinc homeostasis.  Trends in Biochemical Sciences.  August 2003; 28(8): 406-410.  ISSN:  0968-0004
     NAL call no.:   QH345.T73
     Descriptors: prion protein, PrP, transmissible spongiform encephalopathies, TSE, binding of metal ions, protein role may be in zinc concentration management, effects of conformation change.

113. Weiss, D. Beef is back? Rindfleischmarkt auf niedrigerem Niveau stabilisiert.  [Beef is back? Stabilization of the beef market at a lower level.]  May. 2003; 31(1): 25-27.  ISSN:  0341-5155.  In German.
     Descriptors: cattle, beef bulls, level of veal and beef consumption, BSE crisis, producer prices, European Countries, Germany.

114. Wells, Gerald A.H.; Hawkins, Stephen A. C.; Austin, Anthony R.; Ryder, Stephen J.; Done, Stanley H.; Green, Robert B.; Dexter, Ian; Dawson, Midchael; Kimberlin, Richard H. Studies of the transmissibility of the agent of bovine spongiform encephalopathy to pigs. Journal of General Virology. April 2003; 84(4): 1021-1031. ISSN:  0022-1317
     NAL call no.:   QR360.A1J6
     Descriptors: BSE, transmissibility to pigs, parenteral inoculation via intracranial, intravenous, and intraperitoneal routes, incubation period of 69-150 weeks, infectivity testing, bioassay in inbred mice, infectivity found in pig’s stomach, jejunum, distal ileum, pancreas, CNS, orally exposed animals not infected. 

115. Wichert von Holten, S. Psychosoziale Belastungen der Menschen bei Massentoetungen von Nutztieren.  [Psycho-social stress in humans at mass slaughter of farm animals.] DTW Deutsche Tieraerztliche Wochenschrift.  May 2003; 110(5): 196-199. ISSN:  0341-6593.  In German.
     NAL call no.:   41.8 D482
     Descriptors: veterinarians, concerned people, slaughter teams, mass slaughter, contaminated animals, BSE, MKS epidemics, emotional consequences, pastoral care task force service, crisis and stress, Lower Saxony.  

116. Williams, E.S.; Miller, M. W. Transmissible spongiform [encepiharopathies]  (encephalopathies) in non-domestic animals: Origin, transmission and risk factors. Revue Scientifique et Technique Office International des Epizooties. April 2003; 22(1): 145-156.  ISSN:  0253-1933
     NAL call no.:   SF781.R4
     Descriptors: transmissible spongiform encephalopathies, TSE, scrapie, bovine spongiform encephalopathy, BSE, transmissible mink encephalopathy, chronic wasting disease, deer, elk, oral transmission, impact on free ranging cervids, captive animal feed stuffs as sources, recommended surveillance of domestic and wild, infectious agent.

117. Wolferstan, Frances. Slow neurodegeneration and transmissible spongiform encephalopathies/prion diseases Hypothesis: A cycle involving repeated tyrosine kinase A activation could drive the development of TSEs. Medical Hypotheses.  January 2003; 60(1): 52-64. ISSN: 0306-9877
     Descriptors: TSE theory, prion proteins, nerve regeneration and repair, prions modulate tryosine kinase activation, abnormal prion isoforms, damaged and release fragments of prion PrP106-126, stimulate release of nerve growth factor, which activates tyrosine kinase once more, setting up the vicious spiral of slow neurodegeneration found in transmissible spongiform encephalopathies.

118. Ye, X; Carp, R.I.; Meeker, H.C.; Scallet, A.C. Identification and detection of transmissible spongiform encephalopathies.  Current Medicinal Chemistry, Immunology, Endocrine, and Metabolic Agents.  June 2003; 3(2): 95-111.  ISSN:  1568-0134
     Descriptors: TSE, sheep, goats, scrapie, prion disease, ELISA, Western blot, brain biopsy.

2002

1. Abiola, Oduola O.; Iyegbe, Conrad; Lantos, Peter; Plomin, Robert; Anderton, Brian H.; Whatley, Stephen A. Profound sex-specific effects on incubation times for transmission of bovine spongiform encephalopathy to mice. Intervirology. 2002; 45(1): 56-8 ISSN: 0300-5526.
   Abstract: Four strains of mice were inoculated intracerebrally with a primary isolate of bovine spongiform encephalopathy (BSE) and the cloned mouse-adapted scrapie strain ME7. Clinical prion disease diagnosis was made at the appearance of three or more neurological symptoms and their persistence for 3 consecutive weeks and confirmed by neuropathological criteria. For BSE, incubation periods were profoundly different between the sexes in all four mouse strains, being longer in the females. In contrast, ME7 scrapie incubation times were similar between the sexes. Our results indicate that sex-specific processes are involved in the course of primary BSE transmission. Research into this phenomenon may provide clues to the prophylaxis of BSE and have possible implications for new variant Creutzfeldt-Jakob disease in humans.
   NAL call no. QR355.I5
   Descriptors: BSE, scrapie strain ME7, 4 strains of mouse models, inbred C57BL, inbred DBA; experimental infections, brain inoculation, sex differences, NvCreutzfeldt-Jakob Disease, prion physiology.

2. Agerholm, J. S.; Tegtmeier, C. L.; Nielsen, T. K. Survey of laboratory findings in suspected cases of bovine spongiform encephalopathy in Denmark from 1990 to 2000. APMIS, Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 54-60 ISSN: 0903-4641.
   Abstract: A survey of the laboratory findings in suspected cases of bovine spongiform encephalopathy (BSE) in Denmark from 1 June 1990 to 31 December 2000 is presented. During this period BSE was a notifiable disease, and the heads of suspected cases were submitted according to the legislation on BSE. A total of 176 submissions were made, mostly from bovines with neurological disorders and mainly during the last 3 years of this period. Lesions or other laboratory findings consistent with severe neurological disorders were found in 115 cases. The most frequent diagnosis was encephalic listeriosis (35.8% of submissions) followed by other forms of inflammatory lesions. A wide range of lesions were diagnosed less prevalent. BSE was diagnosed twice. The first case occurred in an imported cow in 1992, while the second confirmed case was diagnosed in a native cow in February 2000. A marked increase in the number of submissions occurred following the detection of BSE in February 2000.
   NAL call no. QR1.A6
   Descriptors: cattle, BSE, checking bovines exhibiting neurological disorders, Denmark.

3. Aguzzi, Adriano. Die Prion-Hypothese und Prionen-Krankheiten des Menschen. [The prion hypothesis and the human prion diseases]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 91-8 ISSN: 0005-9366. In German.
   Abstract: Our understanding of the pathogenesis of the transmissible spongiform encephalopathies (TSE) has made terrific headway over the past 40 years and some scientists are even of the opinion that this group of diseases belongs to the neurodegenerative syndromes best understood. On the other hand, the investigation of TSE has led to a multitude of unexpected and surprising results and consequently has initiated impassioned discussions among scientists. Although the human forms of TSE are very rare, the wildfire-like spread of the bovine spongiform encephalopathy (BSE) raises the pressing question as to whether BSE is communicable to humans. This overview summarizes some current hypotheses about the nature of the infectious agent and about the pathogenesis of the damage of the central nervous system.
   NAL call no. 41.8 B45
   Descriptors: pathogenesis, transmissible spongiform encephalopathies, BSE, infectous agent, prion diseases, cattle, humans.

4. Alperovitch, Annick; Will, Robert G. Predicting the size of the vCJD epidemic in France. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 33-36.
   DOI: 10.1016/S1631-0691(02)01410-5
   NAL call no. Q2 C6
   Descriptors: NvCJD, Creutzfeldt-Jakob disease, BSE, France, France/UK ratios of disease, exposure to high risk foods, epidemiology.

5. Anand, P. Public health. Decision-making when science is ambiguous. Science. 2002 Mar 8; 295(5561): 1839. ISSN: 1095-9203.
   NAL call no. 470 SCI2
   Descriptors: BSE, bovine spongiform encephalopathy, prevention and control, public health, disease transmission, policy making, probability.

6. Anonymous. Embryo transfer and BSE. Veterinary Record. London : The British Veterinary Association. Mar 23, 2002. v. 150 (12) p. 357. ISSN: 0042-4900.
   NAL call no. 41.8 V641
   Descriptors: cattle, embryo transfer, BSE, bovine spongiform encephalopathy, disease transmission, UK.

7. Anonymous. Harvard study finds BSE poses little threat to U.S. consumers, agriculture. Journal of the American Veterinary Medical Association. 2002 Feb 1; 220(3): 279-80. ISSN: 0003-1488.
   NAL call no. 41.8 AM3
   Descriptors: animal feed standards, BSE, bovine spongiform encephalopathy, prevention and control, US laws.

8. Aucouturier, Pierre. Dendritic cells and prion propagation. The Scientific World Journal [online] Feb, 2002; 1 (Cited April 4, 2002): 38-40.
   Descriptors: prion protein, movement of protein from the intestinal system, dendretic cells, lymph system.

9. Aupperle, H.; Lucker, E.; Overhoff, M.; Schoon, H. A. Verfahren zum Nachweis von im Hinblick auf die BSE unerwunschten Zutaten in Fleischerzeugnissen: 6. Immunhistologischer Nachweis von zentralem und peripherem Nervengewebe in Fleischerzeugnissen. [Procedures for the unwanted ingredients in meat products with regard to BSE: immunohistochemical procedures for the detection of central and periphere nervous tissue in meat products.] Fleischwirtschaft. 2002., 82: 3, 100-104; 27 Ref. In German with an English summary.
   NAL call no. 280.38 F62
   Descriptors: BSE, bovine spongiform encephalopathy, diagnostic techniques, CNS tissue in muscle tissue, food safety, prevention, quality control.

10. Balen, Adam. Is there a risk of prion disease after the administration of urinary-derived gonadotrophins? Human Reproduction. Oxford. July, 2002; 17 (7): 1676-1680.
    DOI: 10.1093/humrep/17.7.1676-a/
    NAL call no. QP251 H85
    Descriptors: prion protein, scrapie, bovine spongiform encephalopathy, BSE, Creutzfeldt Jakob disease, CJD, contaminated urine in pharmaceuticals, bovine serum.

11. Balter, Michael BSE in sheep? Humiliated lab fights to save face. Science. 2002 Feb 1; 295(5556): 792-3 ISSN: 1095-9203
    NAL call no. 470 SCI2
    Descriptors: brains, sheep, bovine spongiform encephalopathy, cattle, specimen handling, Scotland.

12. Barclay, G. Robin; Houston, E. Fiona; Halliday, Sue I.; Farquhar, Christine F.; Turner, Marc L. Comparative analysis of normal prion protein expression on human, rodent, and ruminant blood cells by using a panel of prion antibodies. Transfusion. 2002 May; 42(5): 517-26. ISSN: 0041-1132.
    Abstract: BACKGROUND: It is not known whether variant CJD can be transmitted within the human population by blood transfusion. The expression of normal cellular prion protein (PrPC) by different blood cell types may permit selective uptake and dissemination of infectivity. STUDY DESIGN AND METHODS: The normal distribution of PrPC on the major blood cell types of species known to be susceptible to natural or experimental transmissible spongiform encephalopathies was studied. Blood from healthy humans, mice, hamsters, cattle, and sheep was examined by flow cytometry by using a large panel of antibodies with different prion protein (PrP) epitope specificities to maximize the detection of PrP variants across species and cell type. RESULTS: PrP was detected on all major human blood cells types except eosinophils, but was not detected as ubiquitously or uniformly on major blood cell types of different animal species. CONCLUSION: Different animal species have unique patterns of expression of PrPC on blood cell types, with none equivalent to the human pattern. This needs to be considered when extrapolating from animal models of blood-borne transmissible spongiform encephalopathy infectivity, particularly in regard to the risk assessment of potential variant CJD spread within the human population. The relationship between PrP distribution and infectivity distribution in blood needs further investigation.
    Descriptors: NvCreuzfeldt-Jakob Disease, prion protein, major blood cells types, humans, C57BL mice hamsters, cattle, sheep, detection of PrP variants, comparison of human results to animals models.

13. Barnicle, D.A. Analysis by Western immunoblotting of differential enzymatic digestion of PrPSc to determine strain of agent. Research in Veterinary Science. April, 2002; 72 (Supplement A): 47. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.8 R312
    Descriptors: prion protein, PrPSc, strain typing, Western blot analysis.

14. Baron, Thierry. Identification of inter-species transmission of prion strains. Journal of Neuropathology and Experimental Neurology. 2002 May; 61(5): 377-83 ISSN: 0022-3069
    Journal URL: http://www.jneuropath.com/pt/re/jnen/home.htm/
    Abstract: The concern of the potential transmission of animal spongiform encephalopathies to humans, which arose as soon as the interspecies transmission of these diseases was recognized, has been reinforced with the emergence of bovine spongiform encephalopathy (BSE) in cattle. Recent experimental findings suggest that the infectious agent causing BSE in cattle can lead to the occurrence of a new form of Creutzfeldt-Jakob disease in humans. These findings help us understand how the transmission to humans of an animal disease may be recognized. This can involve an indirect approach through the analysis of neurodegeneration, either in the disease host, or more specifically, in genetically well-defined experimental hosts to which the disease can be transmitted. Recent experimental studies have also shown that the different molecular features of the abnormal form of the prion protein, which accumulates in the infected tissues, can provide important clues to the relationships between different spongiform encephalopathies. However, a better understanding of the molecular features associated with the specific pathogenic behavior of different strains is required. Complex relationships between the infectious agents involved in spongiform encephalopathies and the disease host can make the recognition of a link between animal prion strains and the human disease difficult to establish.
    Descriptors: cattle, bovine spongiform encephalopathy, prion proteins, molecular features, specific pathogenic behavior of different strains.

15. Beekes, M.; Kurth, R. BSE und Creutzfeldt-Jakob-Krankheit - Gesundheitspolitische Bedeutung fur die Bundesrepublik Deutschland und Europa. [BSE and Creutzfeldt-Jakob disease. Implication on health politics in Germany and Europe]. Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 335-40. ISSN: 0012-0472. In German
    NAL call no. 448.8 D48
    Descriptors: bovine spongiform encephalopathy, prevention, control, epidemiology, transmission, cats, cattle, sheep, Belgium, Germany, France, Europe, meat contamination.

16. Behrens, Axel; Aguzzi, Adriano. Small is not beautiful: Antagonizing functions for the prion protein PrPC and its homologue Dpl. Trends in Neurosciences. March, 2002; 25 (3): 150-154.
    DOI: 10.1016/S0166-2236(00)02089-0
    NAL call no. RC321 T74
    Descriptors: prion conformation variant, doppel protein, homology with PrPC, Dpl is dispensable for prion disease progression, prion biology.

17. Biedermann, Wolfgang; Lucker, Ernst; Hensel, Andreas. Detection of tissues of the central nervous system (CNS) as specified risk material (SRM) in meat products by means of gas chromatography-mass spectrometry (GC-MS). Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 131-3 ISSN: 0005-9366
    Abstract: Determination of specified risk material (SRM) in processed meat products was performed by quantification of brain specific fatty acids using gas chromatography-mass spectrometry (GC-MS). Results from SMP (internal standardised meat products) based analyses showed that absolute concentrations of CNS are correlated (r2 = > 0.97) with the contents of the CNS typical fatty acids docosahexaenoic acid (C 22:6), nervonic acid (C 24:1), lignoceric acid (C 24) and cerebronic acid (C 24oh). GC-MS detection limits were measured at 0.01% CNS. The cut off value was calculated at 0.39% (w/w) CNS in SMP. In a controlled blindfold experiment we were able to identify correctly all positive and negative SMP samples, respectively. Our results indicate that GC-MS based SRM detection may serve as a reference method for immunochemical and immunohistochemical determination of SRM in processed meat products.
    NAL call no. 41.8 B45
    Descriptors: neural tissue contamination, meat products analysis, immunochemical and immunohistochemical determination.

18. Biffiger, Karin; Zwald, Daniel; Kaufmann, Lukas; Briner, Alexandra; Nayki, Inci; Purro, Mario; Bottcher, Sigrid; Struckmeyer, Thomas; Schaller, Olivier; Meyer, Rudolf; Fatzer, Rosemarie; Zurbriggen, Andres; Stack, Mick; Moser, Markus; Oesch, Bruno; Kubler, Eric. Validation of a luminescence immunoassay for the detection of PrP(Sc) in brain homogenate. Journal of Virological Methods. 2002 Mar; 101(1-2): 79-84. ISSN: 0166-0934.
    Abstract: A luminescence immunoassay (LIA) was developed for the diagnosis of bovine spongiform encephalopathy (BSE) in brain tissue using two different monoclonal antibodies for capture and detection of the protease-resistant fragment of the pathological prion protein (PrP27-30). PrP27-30 currently represents the most reliable marker for the infectious particle (denominated prion) causing transmissible spongiform encephalopathies (TSEs). Internal and official validation studies of this assay are described using brain homogenates from ascertained BSE positive and negative cows. Using more than 300 positive and 1400 negative bovine or ovine samples, an excellent sensitivity and specificity of 100% were demonstrated. More than 1000-fold dilutions of a BSE positive homogenate still resulted in a clear positive signal. In combination with a simple homogenisation procedure for the preparation of the samples, this assay lends itself for large scale screening of cattle and sheep for TSEs using complete automation of the process.
    NAL call no. QR355.J6
    Descriptors: BSE, brain tissue analysis, cattle, diagnostic test, luminescense immunoassay, brain homogenates, screening of cattle and sheep for transmissible spongiform encephalopathies.

19. Bingham, Brian. New variant CJD-BSE (mad cow disease). The need for disposable ENT instruments. International Journal of Pediatric Otorhinolaryngology. 2002 Feb 25; 62(3): 203-6. ISSN: 0165-5876.
    Abstract: This paper outlines the development of Bovine Spongiform Encephalopathy (BSE) in the United Kingdom. The relationship between BSE and new variant Creutzfeldt-Jakob disease (vCJD) is considered and the risks of iatrogenic spread reviewed. The rationale for disposable surgical instruments in adenotonsillectomy to prevent iatrogenic spread is discussed.
    Descriptors: BSE, bovine spongiform encephalopathy, iatrogenic spread, surgical instruments, risks of transmission, UK.

20. Blattler, Thomas. Transmission of prion disease. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 71-8. ISSN: 0903-4641
    Abstract: The transmission of bovine spongiform encephalopathy to humans as variant Creutzfeldt-Jakob disease (vCJD) has focused public attention on how prion diseases are transmitted and how prions reach the brain after exposure. Prion diseases are characterised by transmissibility and neuropathological features of gliosis, neuronal loss and microscopic vacuoles, termed spongiosis. The principal component of prions is the glycoprotein PrP(Sc), which is a conformational modified isoform of the normal membrane protein PrP(C). How are prions transmitted and how do prions find their way once they have been ingested? Prion models in mouse and hamster point to lymphoreticular cells which support an early replication phase of prions before reaching the central nervous system via peripheral nerves. Whilst some key players seem to have been identified so far, the mechanisms of prion propagation to the brain are still not fully understood. Seemingly contradictory results have led to some confusion and have provoked discussion.
    NAL call no. QR1.A6
    Descriptors: BSE, spongiform encephalopathies, transmission, Nv Creutzfeldt-Jakob Disease, path to brain after exposure, mouse and hamster animal models, pmyphoreticular cells, peripheral nerve pathway, discussion of mechanism of prion propagation.

21. Bons, Noelle; Lehmann, Sylvain; Mestre, Frances-Nadine; Dormont, Dominique; Brown, Paul Brain and buffy coat transmission of bovine spongiform encephalopathy to the primate Microcebus murinus. Transfusion. May, 2002; 42 (5): 513-516.
    Descriptors: BSE, NvCreutzfeldt-Jacob Disease, secondary transmission, blood and blood products, macaque monkeys, Microcebus murinus, challenged with brain homogenate and buffy coat, transmission of infection.

22. Bons, Noelle; Lehmann, Sylvain; Nishida, Noriyuki; Mestre, Frances Nadine; Dormont, Dominique; Belli, Patrick; Delacourte, Andre; Grassi, Jacques; Brown, Paul. BSE infection of the small short-lived primate Microcebus murinus. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 67-74.
    DOI: 10.1016/S1631-0691(02)01390-2
    NAL call no: Q2 C6
    Descriptors: Microcebus murinus (lemur) primates, disease susceptibility, intracerebrally/orally infected prion proteins, BSE /macaque-adapted BSE brain inoculates, hyperaggregated and paired-helical filaments-immunoreactive Tan proteins, beta42-amyloid plaques and astrogliosis, PrPres.

23. Boratynski, J.; Gorski, A. BSE: A consequence of cattle feeding with glycated molecules host-unknown? Medical Hypotheses. April, 2002; 58 (4): 276-278.
    Descriptors: pathogenesis, BSE, transmissible spongiform encephalopathies, scrapie, sheep, goats, cattle, disease origins, glycated proteins in feeds, high temperature glycation process.

24. Borchers, Kerstin. Transmissible Spongiforme Enzephalopathien (TSE): Alte Krankheiten mit neuer Brisanz. [Transmissible spongiform encephalopathies (TSE): old diseases with new explosive force]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 81-90 ISSN: 0005-9366. In German.
    Abstract: In view of the first 64 BSE cases (date: 11.5.01) in German cattle herds an overview on TSE and their similarities and differences regarding clinic, pathogenesis and pathology is given. The mechanism of the unconventional agent, an infectious protein (prion), is explained based on the prion model of Stanley Prusiner. The knowledge on transmission, incubation time, host specificity as well as resistance and immunity drawn from experimentally infected animals is discussed. Thus, after oral infection prions are transported by lymphocytes from the stomach-intestinal tract to the spleen. The way to the CNS is still unknown. The presumption for crossing the species barrier is twofold: first the prions of different species have to be biochemically homologous and a genetical disposition has to exist. This is the case for BSE and the new variant of Creutzfeldt-Jakob-Disease (vCJD). There is evidence that in Great Britain so far 97 (date: 30.3.01) young people acquired vCJD due to consumption of food that contained bovine risk material. Regarding the infectious prion dosis brain, spinal cord and lymphoid tissues are regarded to be most dangerous. The principle of the BSE-test, its evidence as well as steps for prevention and control of BSE are presented.
    NAL call no. 41.8 B45
    Descriptors: cattle, transmissible spongiform encephalopathies, comparisons, pathogenesis, pathology, prion proteins, incubation time, immunity, vCreutzfeldt-Jakob Disease, Germany.

25. Bosque, Patrick J.; Ryou, Chongsuk; Telling, Glenn; Peretz, David; Legname, Giuseppe; DeArmond, Stephen J.; Prusiner, Stanley B. Prions in skeletal muscle. Proceedings of the National Academy of Sciences of the United States of America. 2002 Mar 19; 99(6): 3812-7. ISSN: 0027-8424.
    Abstract: Considerable evidence argues that consumption of beef products from cattle infected with bovine spongiform encephalopathy (BSE) prions causes new variant Creutzfeldt-Jakob disease. In an effort to prevent new variant Creutzfeldt-Jakob disease, certain "specified offals," including neural and lymphatic tissues, thought to contain high titers of prions have been excluded from foods destined for human consumption [Phillips, N. A., Bridgeman, J. & Ferguson-Smith, M. (2000) in The BSE Inquiry (Stationery Office, London), Vol. 6, pp. 413-451]. Here we report that mouse skeletal muscle can propagate prions and accumulate substantial titers of these pathogens. We found both high prion titers and the disease-causing isoform of the prion protein (PrP(Sc)) in the skeletal muscle of wild-type mice inoculated with either the Me7 or Rocky Mountain Laboratory strain of murine prions. Particular muscles accumulated distinct levels of PrP(Sc), with the highest levels observed in muscle from the hind limb. To determine whether prions are produced or merely accumulate intramuscularly, we established transgenic mice expressing either mouse or Syrian hamster PrP exclusively in muscle. Inoculating these mice intramuscularly with prions resulted in the formation of high titers of nascent prions in muscle. In contrast, inoculating mice in which PrP expression was targeted to hepatocytes resulted in low prion titers. Our data demonstrate that factors in addition to the amount of PrP expressed determine the tropism of prions for certain tissues. That some muscles are intrinsically capable of accumulating substantial titers of prions is of particular concern. Because significant dietary exposure to prions might occur through the consumption of meat, even if it is largely free of neural and lymphatic tissue, a comprehensive effort to map the distribution of prions in the muscle of infected livestock is needed. Furthermore, muscle may provide a readily biopsied tissue from which to diagnose prion disease in asymptomatic animals and even humans.
    NAL call no. 500 N21P
    Descriptors: BSE, infected beef products, NvCreutzfeldt-Jacob disease, mouse model, skeletal muscle accumulations, PrPSc, Me7, Rocky Mountain Laboratory strain murine prions, possible diagnostic tissue for testing.

26. Bousset, Luc; Melki, Ronald. Similar and divergent features in mammalian and yeast prions. Microbes and Infection. Institut Pasteur 2002 Apr; 4(4): 461-9. ISSN: 1286-4579.
    Abstract: Mammalian transmissible spongiform encephalopathies are likely due to the propagation of an abnormal form of a constitutive protein instead of traditional genetic material (nucleic acids). Such infectious proteins, which are termed prions, exist in yeast. They are at the origin of a number of phenotypes that are inherited in a non-Mendelian manner. These prions are very useful to dissect the molecular events at the origin of this structure-based inheritance. The properties of mammalian and yeast prions are presented and compared. This review highlights a number of similarities and differences.
    NAL call no. QR180 M53
    Descriptors: TSEs, transmissible spongiform encephalopathies, infectious proteins, prions, yeast and mammalian prions compared, a review.

27. Boyce, Nell. The madness of the elk. US News and World Report. 2002 Mar 25; 132(9): 56. ISSN: 0041-5537.
    NAL call no. 280.8 Un33A
    Descriptors: animal diseases, etiology, wild deer, epidemiology, transmissible spongiform encephalopathy, BSE, USA.

28. Bradley, Ray. Bovine spongiform encephalopathy update. Polish Journal of Pathology Official Journal of the Polish Society of Pathologists 2002; 53(1): 7-16. ISSN: 1233-9687.
    Abstract: Bovine spongiform encephalopathy (BSE) is a zoonosis being the origin of variant Creutzfeldt-Jakob disease and an important cattle disease in its own right. Countries have been slow to learn the importance of protecting, not only their cattle populations, but also their human populations. Since 2000, several additional European countries have reported BSE in native-born stock and this has led to a concern about the BSE status of countries that have imported cattle and cattle products from infected countries. Extensive feed and offal bans and application of newly-developed, "Rapid" tests for prion protein in central nervous tissue of targeted, high-risk animals and slaughter cattle over 30 months old now provides the tools whereby the public are fully protected and BSE can be eradicated.
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, cattle, human health risks, prion protein testing in slaughtered animals, control, eradication.

29. Bren, Linda. FDA continues work to help prevent mad cow disease. FDA Consumer. 2002 May-Jun; 36(3): 31-2. ISSN: 0362-1332
    NAL call no. HD9000.9 U5A1
    Descriptors: BSE, bovine spongiform encephalopathy, transmission prevention and control, U.S. Food and Drug Administration, sentinel and surveillance, cattle, animal feeds, U.S.

30. Brenig, Bertram; Schutz, Ekkehard; Urnovitz, Howard. Zellulare Nucleinsauren im Serum und Plasma als neue diagnostische Werkzeuge. [Cellular nucleic acids in serum and plasma as new diagnostic tools]. Berliner und Munchener tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 122-4 ISSN: 0005-9366. In German.
    Abstract: Currently, the diagnosis of bovine spongiform encephalopathy is only possible in the brain stem of dead animals. Protease resistant prions are detected in the obex region of the brain stem. However, from a veterinary medical and agricultural point of view the development of an in vivo detection assay is of utmost importance. Because infectious prions are detectable relatively late in the central nervous system during an infection, efforts are made searching for surrogate markers. Besides neuronal proteins that are released into the liquor and blood during neurodegenerative processes or other neuronal diseases, cellular nucleic acids circulating in the plasma or serum are an absolutely new approach for the detection of infectious diseases.
    NAL call no. 41.8 B45
    Descriptors: BSE, bovine spongiform encephalopathy, vivo detection assay, new approach, cellular nucleic acids in plasma or serum.

31. Breslin, P. D.; Bassett, H. F.; McElroy, M.C.; Markey, B. K.; Weavers, E. Brainstem cell populations in BSE-affected and clinically normal cattle. Research in Veterinary Science. April 2002; 72 (Supplement A): 46. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: cattle, bovine spongiform encephalopathy, brainstem cells, comparison study, diseased and normal animals.

32. Brown, David R Mayhem of the multiple mechanisms: modelling neurodegeneration in prion disease. Journal of Neurochemistry. 2002 Jul; 82(2): 209-15 ISSN: 0022-3042.
    Abstract: This review examines recent attempts to advance the understanding of the mechanism by which neurones die in prion disease. Prion diseases or transmissible spongiform encephalopathies are characterized by the conversion of a normal glycoprotein, the prion protein, to a protease-resistant form that is suggested to be both the infectious agent and the cause of the rapid neurodegeneration in the disease. Death of the patient results from this widespread neuronal loss. Thus understanding the mechanism by which the abnormal form of the prion protein causes neuronal death might lead to treatments that would prevent the life-threatening nature of these diseases.
    NAL call no. QP351.J6
    Descriptors: prion diseases, neuronal death patterns, animal models, physiopathology, transmissible spongiform encephalopathies.

33. Brown, David R. Molecular advances in understanding inherited prion diseases. Molecular Neurobiology. June, 2002; 25 (3): 287-302.
    NAL call no. QP365.2 M64
    Descriptors: BSE, bovine spongiform encephalopathy, variant Creutzfeldt Jakob disease, CJD.

34. Brown, Paul. Drug therapy in human and experimental transmissible spongiform encephalopathy. Neurology. 2002 Jun 25; 58(12): 1720-5. ISSN: 0028-3878.
    Abstract: During the past 30 years, over 60 different chemical compounds have been used to treat experimental animals infected with transmissible spongiform encephalopathies (TSE), including a wide variety of anti-infectious agents, immunomodulating drugs, and chemicals interacting with the lympho-reticular system. Some compounds achieved a prolongation of the incubation period, but this effect decreased or disappeared when they were administered at or near the onset of symptomatic disease. Recent in vitro and tissue culture studies support earlier speculation about the importance of a chemical structure containing both water-soluble and lipid-soluble components, evidently as a means of interaction with the misfolded membrane-bound 'prion' protein. A number of compounds shown to eliminate the protein (or infectivity) in TSE-infected tissue cultures are the subject of ongoing studies in animals, and are under consideration for human drug trials. As with other recalcitrant infections, combinations of drugs with different modes of action are likely to be necessary for any effective therapy. Also, very recent work in developing antibodies that can neutralize in vitro infection (and, in conjunction with genetic engineering, in vivo infection) has renewed interest in the strategies of both active and passive immunization.
    Descriptors: drugs, treatments, TSEs, anti-infectious agents, immunomodulation drugs, chemicals, experimental animals, prion diseases, prion proteins.

35. Bruce, Moira E.; Boyle, Aileen; Cousens, Simon; McConnell, Irene; Foster, James; Goldmann, Wilfred; Fraser, Hugh Strain characterization of natural sheep scrapie and comparison with BSE. Journal of General Virology. 2002 Mar; 83(Pt 3): 695-704 ISSN: 0022-1317.
    Abstract: Scrapie was transmitted to mice from ten sheep, collected in the UK between 1985 and 1994. As in previous natural scrapie transmissions, the results varied between scrapie sources in terms of the incidence of disease, incubation periods and neuropathology in challenged mice. This contrasted with the uniformity seen in transmissions of BSE to mice. The scrapie and BSE isolates were characterized further by serial passage in mice. Different TSE strains were isolated from each source according to the Sinc or PrP genotype of the mouse used for passage. The same two mouse-passaged strains, 301C and 301V, were isolated from each of three BSE sources. Despite the variation seen in the primary transmissions of scrapie, relatively few mouse-passaged scrapie strains were isolated and these were distinct from the BSE-derived strains. The ME7 scrapie strain, which has often been isolated from independent sheep sources in the past, was identified in isolates from four of the sheep. However, a new distinct strain, 221C, was derived from a further four scrapie sheep. These results suggest that there is agent strain variation in natural scrapie in sheep and that the spectrum of strains present may have changed over the last 20 years. The tested sample is too small to come to any conclusions about whether the BSE strain is present in sheep, but the study provides a framework for further more extensive studies.
    NAL call no. QR360.A1J6
    Descriptors: scrapie, transmitted to mice, BSE isolates, serial passage in mice, ME7 strain, 221C strain, strain evolution.

36. Brufau, J.; McCartney, E. 'Feed scares': where is Europe leading? Feed International. 2002, 23: 2, 6-8.
    Descriptors: animal feeds, transmissible spongiform encephalopathies, livestock animals, epidemiology, control, Europe.

37. Bucciantini, Monica; Giannoni, Elisa; Chiti, Fabrizio; Baroni, Fabiana; Formigli, Lucia; Zurdo, Jesus; Taddei, Niccolo; Ramponi, Giampietro; Dobson, Christopher M.; Stefani, Massimo. Inherent toxicity of aggregates implies a common mechanism for protein misfolding diseases. Nature. April 4, 2002; 416 (6880): 507-11. ISSN: 0028-0836
    DOI: 10.1038/416507a
    Abstract: A range of human degenerative conditions, including Alzheimer's disease, light-chain amyloidosis and the spongiform encephalopathies, is associated with the deposition in tissue of proteinaceous aggregates known as amyloid fibrils or plaques. It has been shown previously that fibrillar aggregates that are closely similar to those associated with clinical amyloidoses can be formed in vitro from proteins not connected with these diseases, including the SH3 domain from bovine phosphatidyl-inositol-3'-kinase and the amino-terminal domain of the Escherichia coli HypF protein. Here we show that species formed early in the aggregation of these non-disease-associated proteins can be inherently highly cytotoxic. This finding provides added evidence that avoidance of protein aggregation is crucial for the preservation of biological function and suggests common features in the origins of this family of protein deposition diseases.
    NAL call no. 472 N21
    Descriptors: spongiform encephalopathies, proteinaceous aggregates, fibrillar aggregates, bovine phosphatidylinositol-3-kinase chemistry. PC12 cells, rats.

38. Budka, Herbert; Dormont, Dominique; Kretzschmar, Hans; Pocchiari, Maurizio; van-Duijn, Cornelia.. BSE and variant Creutzfeldt-Jakob disease: never say never. Acta Neuropathologica 2002 Jun; 103(6): 627-8 ISSN: 0001-6322
    Descriptors: bovine spongiform encephalopathy, NvCJD, prion pathogenicity, epidemiology, animal models, transmission and control.

39. Busk, N.; Vaughan, K.; Watkins, R.; Hawkins-S.A.C. Review and refinement of clinical monitoring methods for TSE mouse bioassay/strain typing studies. Research in Veterinary Science. April, 2002; 72 (Supplement A): 46-47. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.8 R312
    Descriptors: transmissible spongiform encephalopathies, mouse model bioassay, strain typing, prions.

40. Cai, Kang; Miller, Jeanette L.C.; Stenland, Christopher J.; Gilligan, Kevin J.; Hartwell, Randal C.; Terry, Jarrett C.; Evans-Storms, Rosemary B.; Rubenstein, Richard; Petteway, Stephen R. Jr; Lee, Douglas C. Solvent-dependent precipitation of prion protein. Biochimica et Biophysica Acta - Protein Structure and Molecular Enzymology. 20 May, 2002; 1597 (1): 28-35. ISSN: 0006-3002.
    NAL call no. 381 B522
    Descriptors: misfolded isoform, prion protein, PrPSc, solvents, physiological buffer, pH effects, salt, ethanol concentration.

41. Carlson, George A. Postexposure prophylaxis against transmissible spongiform encephalopathies: CpG oligodeoxynucleotides in mice. Lancet. 2002 Jul 20; 360(9328): 184. ISSN: 0140-6736.
    NAL call no. 449.8 L22
    Descriptors: cattle, adjuvants, immunologic therapeutic use, BSE, control, mouse models, oligodeoxyribonucleotides.

42. Carruthers, Jean; Carruthers, Alastair, Mad cows, prions, and wrinkles. Archives of Dermatology. 2002 May; 138(5): 667-70 ISSN: 0003-987X.
    NAL call no. 448.8 AR242
    Descriptors: BSE transmission risks, collagen, prion physiology, prion disease prevention and control, cosmetic formulations, injections, intradermal.

43. Chamberland, Mary E. Emerging infectious agents: do they pose a risk to the safety of transfused blood and blood products? Clinical Infectious Diseases, Official Publication of the Infectious Diseases Society of America. 2002 Mar 15; 34(6): 797-805. ISSN: 1537-6591.
    Abstract: The blood supply is safer than it has been at any other time in recent history, and, in the context of other health care-related adverse events, the risks associated with blood transfusion are extremely small. The current high level of safety is the result of successive refinements and improvements in how blood is collected, tested, processed, and transfused; nonetheless, blood and plasma products remain vulnerable to newly identified or reemerging infections. In recent years, numerous infectious agents-including several newly discovered hepatitis viruses, the agents of transmissible spongiform encephalopathies, and tickborne pathogens-have been identified as potential threats to the safety of blood and plasma. Continued vigilance is critical to protect the blood supply from known pathogens and to monitor for the emergence of new infectious agents. Recent terrorist activities in the United States add new considerations to maintaining the safety and supply of blood. Education of clinicians and patients regarding the benefits and risks associated with the judicious use of blood and blood products can assist in informed decision making.
    NAL call no. RC111 R4
    Descriptors: blood supply, safety risks, transmissible spongiform encephalopathies, recommendations for decision making.

44. Chaplin, M.J.; Barlow, N.; Ryder, S.; Simmons, M.M.; Spencer, Y.; Hughes, R.; Stack, M. J. Evaluation of the effects of controlled autolysis on the immunodetection of PrPSc by immunoblotting and immunohistochemistry from natural cases of scrapie and BSE. Research in Veterinary Science. February, 2002; 72 (1): 37-43.
    NAL call no. 41.8 R312
    Descriptors: sheep, cattle, scrapie, BSE, histopathological examination, medulla brain exam, Western immunoblotting for detection of PrPSc, Prionics diagnostic test, routine immunohistochemical technique.

45. Cochrane, N. Pressures for change in Eastern Europe's livestock sectors. Agricultural Outlook. 2002, No.288, 17-20.
    NAL call no. aHD1751.A42
    Descriptors: Central Europe, disease control, transmissible spongiform encephalopathy, livestock testing, trade, disease prevention and control.

46. Cooper, J.E. Diagnostic pathology of selected diseases in wildlife. Revue Scientifique et Technique Office International des Epizooties. April, 2002; 21 (1): 77-89.
    URL: www.oie.int/eng/publicat/rt/2101/A_R2115.htm
    NAL call no. SF781 R4
    Descriptors: wildlife, detection, management, infectious disease, field diagnosis, sampling, principles, diagnostic pathology, mycobacteriosis, Rift Valley fever, rabies, spongiform encephalopathies, morbillivirus and poxvirus infections, viral encephalitides, West Nile virus infection and chytridiomycosis.

47. Croes, E.A.; van Gool, W.A.; Jansen, G. H.; van Duijn, C. M. Ziekte van Creutzfeldt-Jakob: diagnostiek, incidentie, preventie en behandeling. [Creutzfeldt-Jakob disease: diagnosis, incidence, prevention and treatment]. Nederlands Tijdschrift voor Geneeskunde 2002 Apr 20; 146(16): 750-4. ISSN: 0028-2162. In Dutch.
    Abstract: Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative disorder belonging to the spongiform encephalopathies. A variant form (vCJD) is most likely the result of infection with the agent that causes bovine spongiform encephalopathy (BSE). Diagnostic information can be obtained by EEG, testing cerebrospinal fluid for the presence of the 14-3-3 protein, MRI, brain biopsy, tonsil biopsy, and postmortem brain examination. Some tests, such as MRI and postmortem brain examination, can be used to distinguish between CJD and vCJD. Pathological prions in a tonsil biopsy are only found with vCJD. In the Netherlands, there are four known cases of iatrogenic CJD. On the basis of certain exposure to BSE via the food chain, cases of vCJD are also to be expected. Chloropromazine and mepacrine are known to inhibit the formation of pathological prion conformations, but clinical trials have not yet been carried out.
    Descriptors: NvCJD, bovine spongiform encephalopathy, BSE, diagnosis of CJD and NvCJD, humans, iatrogenic disease, MRI, brain biopsy, tonsil biopsy, postmortem brain examination, chloropromazine, mepacrine, treatment.

48. Dalsgaard, Niels Jorn. Prion diseases. An overview. APMIS Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 3-13. ISSN: 0903-4641.
    Abstract: Prion disease is the new designation of a group of spongiform encephalopathies, all invariably fatal, which show similar clinical and neuropathological changes. They comprise a range of distinct diseases in both animals and man, and spontaneous, hereditary and transmissible forms are recognized. Until the sudden occurrence in the mid-1980s of an epizootic of a formerly unknown disease, popularly named 'mad cow disease', in cattle in the UK, very little attention had been paid to these rather obscure diseases. Concurrently it was asserted that the disease-causing agent appeared to be a ubiquitous mammalian brain constituent, and the disease mechanism a conformational change of its structure. These events have not only led to a new understanding of these extraordinary diseases, but have also provided insight into both neurodegeneration and disease mechanisms at the molecular level. Moreover, in 1997 the prion concept earned its originator the second Nobel price for medicine within this scientific field. In this introduction and overview of prion diseases, historical and philosophical perspectives are presented along with descriptions of the diseases in both animals and man. Epidemiology, genetics and transmissibility are also covered.
    NAL call no. QR1.A6
    Descriptors: spongiform encephalopathies, BSE, bovine spongiform encephalopathies, neurodegeneration, disease mechanisms, prions, overview, history, descriptions of diseases, epidemiology, genetics, transmissibility, US, UK.

49. Daly, D. J.; Prendergast, D. M.; Sheridan, J. J.; Blair, I.S.; McDowell, D.A. Use of a marker organism to model the spread of central nervous system tissue in cattle and the abattoir environment during commercial stunning and carcass dressing. Applied and Environmental Microbiology. 2002 Feb; 68(2): 791-8. ISSN: 0099-2240.
    Abstract: Due to concerns about a link between variant Creutzfeldt-Jakob disease in humans and similar prion protein-induced disease in cattle, i.e., bovine spongiform encephalopathy (BSE), strict controls are in place to exclude BSE-positive animals and/or specified risk materials including bovine central nervous system (CNS) tissue from the human food chain. However, current slaughter practice, using captive bolt guns, may induce disruption of brain tissues and mobilize CNS tissues into the bovine circulatory system, leading to the dispersion of CNS tissues (including prion proteins) throughout the derived carcass. This project used a marker (antibiotic-resistant) strain of Pseudomonas fluorescens to model the effects of commercial captive bolt stunning procedures on the movement of mobilized CNS material within slaughtered animals and the abattoir environment. The marker organism, introduced by injection through the bolt entry aperture or directly using a cartridge-fired captive bolt, was detected in the slaughter environment immediately after stunning and in the abattoir environment at each subsequent stage of the slaughter-dressing process. The marker organism was also detected on the hands of operatives; on slaughter equipment; and in samples of blood, organs, and musculature of inoculated animals. There were no significant differences between the results obtained by the two inoculation methods (P < 0.05). This study demonstrates that material present in, or introduced into, the CNS of cattle during commercial captive bolt stunning may become widely dispersed across the many animate and inanimate elements of the slaughter-dressing environment and within derived carcasses including meat entering the human food chain.
    NAL call 448.3 AP5
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, bovine spongiform encephalopathy, cattle, control and prevention of prion disease, slaughter, Pseutomonas fluorescens to model captive bolt stunning, facility monitoring, carcass contamination.

50. Dealler, Steve. vCJD: the epidemic that never was. Possibility of BSE being cause of variant CJD is indeed biologically plausible. BMJ Clinical Research Ed 2002 Jul 13; 325(7355): 102. ISSN: 1468-5833.
    Descriptors: cattle, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Syndrome transmission, contaminated meat, public health risks.

51. Debeer, Sabine O. S.; Baron, Thierry G.M.; Bencsik, Anna A. Transmissible spongiform encephalopathy diagnosis using PrPsc immunohistochemistry on fixed but previously frozen brain samples. Journal of Histochemistry and Cytochemistry. The official journal of the Histochemistry Society 2002 May; 50(5): 611-6. ISSN: 0022-1554.
    URL: www.jhc.org/cgi/content/abstract/50/5/611
    Abstract: The histological diagnosis of transmissible spongiform encephalopathies (TSEs), such as scrapie and bovine spongiform encephalopathy (BSE), relies on identification in the brain of spongiosis, gliosis, and neuron loss without inflammatory lesions. Because of its sensitivity, immunohistochemistry of abnormal prion protein (PrPsc) is of great help in this diagnosis and can be used on its own or complementary to the biochemical detection of PrPsc. However, in some cases no formalin-fixed material is available, rendering its use as a complementary method impossible. For that purpose, we studied the possibility of detecting PrPsc immunohistochemically in fixed brain samples that had been previously frozen and used for Western blotting analysis. We compared freshly and fixed-frozen brain samples originating from the same sheep, either affected or unaffected with scrapie. We also studied fixed-frozen brain samples from scrapie-affected goats and from cows showing BSE. We showed that in all the species tested, despite damage to the histological structures, PrPsc was still detectable in the fixed-frozen brain sections without unspecific background staining. Notwithstanding the limited number of cases thus far analyzed, we have already demonstrated the possibility of using PrPsc immunohistochemistry on fixed-frozen brain samples with very good efficacy, thus rendering possible its use for diagnostic purposes in TSEs.
    NAL call no. 381 J8222
    Descriptors: scrapie, BSE, immunohistochemistry of abnormal prion protein, PrPsc, diagnosis, compared fresh and frozen brain tissue, diagnostic method.

52. Dedet,V. BSE screeningsprover pa levende dyr: tre vaesentlige eksempler pa videnskabelige undersogelser. [BSE screening for live animals. Three good examples of scientific experiments.] Dansk Veterinaertidsskrift. 2002, 85: 1, 18-19. In Danish.
    NAL call no. 41.9 D23
    Descriptors: cattle, sheep, bovine spongiform encephalopathy, diagnostic methods.

53. Delgado-Hachmeister, J.E.; Rangel-Frausto, M.S.; Ponce de Leon, S; de Leon, S. Ponce. Encefalopatias espongiformes transmisibles. [Transmissible spongiform encephalopathies.] Salud Publica de Mexico. 2002, 44: 1, 69-75; 22 ref. In Spanish with an English summary.
    Descriptors: TSE, public health concerns, Mexico.

54. Dickmeiss, Ebbe; Gerstoft, Jan. Blood infectivity in transmissible spongiform encephalopathies. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 99-103 ISSN: 0903-4641.
    Abstract: Blood infectivity in transmissible spongiform encephalopathies (TSE) is reviewed with special emphasis on transmission by blood transfusion in human beings. It is concluded that transmission by transfusion seems biologically plausible as regards variant Creutzfeld-Jakob Disease (vCJD), albeit present knowledge suggests that it is extremely uncommon. Precautionary measures against the putative risk of vCJD transmission by blood transfusion are discussed.
    NAL call no. QR1.A6
    Descriptors: blood products, transmissibility concerns, transmissible spongiform encephalopathies, emphais on humans, NvCreutzfeldt-Jakob Disease, risks.

55. Docampo, Roberto. New and re-emerging infectious diseases. Trends in Parasitology. August, 2002; 18 (8): 334-336. The 5th Annual Conference on New and Re-emerging Infectious Diseases, Urbana-Champaign, IL, USA, April 18-19, 2002.
    DOI: 10.1016/S1471-4922(02)02351-6
    NAL call no. QL757 P374
    Descriptors: transmissible spongiform encephalopathies, various diseases are addressed, zoonotic and animal diseases.

56. Dodd, Roger Y.; Busch, Michael P. Animal models of bovine spongiform encephalopathy and vCJD infectivity in blood: two swallows do not a summer make. Transfusion. 2002 May; 42(5): 509-12. ISSN: 0041-1132.
    Descriptors: Creutzfeldt Jakob Syndrome, transmission, BSE, CJD, bovine spongiform spongiform encephalopathy, animals models, blood transfusion research, lemur, Macaca fascicularis, phosphoprotein-phasphatase analysis, sheep, species specificity.

57. Doherr, M.G. Aetiologie, Uebertragung und epidemiologische Situation von BSE in Europa. [Etiology, transmission and epidemiological situation of BSE in Europe.] Praktische Tierarzt 2002 vol. 83, no. 2, pp. 156-161. In German.
    NAL call no. 41.8 P882
    Descriptors: BSE, European status, epidemiology, etiology, public health concerns, transmissible spongiform encephalopathies.

58. Doherr, M.G.; Hett, A.R.; Cohen, C.H.; Fatzer, R.; Rufenacht, J.; Zurbriggen, A.; Heim, D. Trends in prevalence of BSE in Switzerland based on fallen stock and slaughter surveillance. Veterinary Record. London : The British Veterinary Association. Mar 16, 2002. v. 150 (11) p. 347-348. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, disease prevalence, trends, disease surveys, Switzerland.

59. Domingo, Jose L. Lack of experimental studies on human transmission of BSE in relation with the consumption of specified risk materials (SRM): The case of the milk. Preventive Medicine. June, 2002; 34 (6): 655-656.
    DOI: 10.1006/pmed.2002.1027
    NAL call no. RA421 P684
    Descriptors: BSE, bovine spongiform encephalopathy, transmissible spongiform encephalopathy, public health risks, animal derived foods, milk, research recommendations.

60. Eaton, S.L.; Foster, J. D.; Hunter, N. Follicular dendritic cell involvement in ovine scrapie. Research in Veterinary Science. April, 2002; 72 (Supplement A): 44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
    NAL call no. 41.88 R312
    Descriptors: sheep, scrapie, prion disease, effects on follicular dendritic cells.

61. Eberl, Heike; Glockshuber, Rudi. Folding and intrinsic stability of deletion variants of PrP(121-231), the folded C-terminal domain of the prion protein. Biophysical Chemistry. 2 May, 2002; 96 (2-3): 293-303.
    DOI: 10.1016/S0301-4622(02)00015-7
    NAL call no. QP1.B5
    Descriptors: transmissible spongiform encephalopathies, prions, PrPSc, PrPC structure, deletion variants of C terminal PrPC domain, tertiary structure context, conformation of the segment comprising alpha-helix 2 and 3 in the solution structure of recombinant PrP.

62. Farrugia, Albert. Laboratory practice and studies of bovine spongiform encephalopathy. Lancet. 2002 Mar 23; 359(9311): 1067-8. ISSN: 0140-6736.
    NAL call no. 448.4 L22
    Descriptors: bovine spongiform encephalopathy transmission, laboratory standards, cattle, sheep.

63. Fatzer, R.; Vandevelde, M.; Gottstein, B. Cerebral taeniid oncospheral lesions in two BSE suspects. Veterinary Record. London : The British Veterinary Association. Jan 12, 2002. v. 150 (2) p. 46-47. ISSN: 0042-4900.
    NAL call no. 41.8 V641
    Descriptors: cattle, brain lesions, oncospheres, symptoms, BSE, bovine spongiform encephalopathy, histopathology, Taenia saginata, case report, Switzerland.

64. Ferguson, N.M.; Ghani, A.C.; Donnelly, C.A.; Hagenaars, T.J.; Anderson, R.M. Estimating the human health risk from possible BSE infection of the British sheep flock. Nature. London : Macmillan Magazines Ltd. Jan 24, 2002. v. 415 (6870) p. 420-424. ISSN: 0028-0836
    DOI: 10.1038/nature709
    Abstract: Following the controversial failure of a recent study and the small numbers of animals yet screened for infection, it remains uncertain whether bovine spongiform encephalopathy (BSE) was transmitted to sheep in the past via feed supplements and whether it is still present. Well grounded mathematical and statistical models are therefore essential to integrate the limited and disparate data, to explore uncertainty, and to define data-collection priorities. We analysed the implications of different scenarios of BSE spread in sheep for relative human exposure levels and variant Creutzfeldt-Jakob disease (vCJD) incidence. Here we show that, if BSE entered the sheep population and a degree of transmission occurred, then ongoing public health risks from ovine BSE are likely to be greater than those from cattle, but that any such risk could be reduced by up to 90% through additional restrictions on sheep products entering the food supply. Extending the analysis to consider absolute risk, we estimate the 95% confidence interval for future vCJD mortality to be 50 to 50,000 human deaths considering exposure to bovine BSE alone, with the upper bound increasing to 150,000 once we include exposure from the worst-case ovine BSE scenario examined.
    NAL call no. 472 N21
    Descriptors: cattle, BSE, bovine spongiform encephalopathy, infections, sheep, disease transmission. human diseases, zoonosis, public health, NvCreutzfeldt Jakob Disease, ovine BSE, statistical models, UK.

65. Ferguson-Smith, Malcolm A. Reaction to the emergence of BSE in the UK: What was done and what perhaps might have been done better. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 25-26.
    DOI: 10.1016/S1631-0691(02)01395-1
    NAL call no. Q2 C6
    Descriptors: BSE, bovine spongiform encephalopathy, the UK epidemic, governmental actions, historical perspectives.

66. Fernie, K. Heat/hydroxide combinations as novel decontamination methods for surgical instruments. Research in Veterinary Science. April, 2002; 72 (Supplement A): 43-44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: transmissible spongiform encephalopies, prions, potential contamination of surgical instruments, decontamination methods.

67. Foster, J.D.; Parnham, D.W.; Hunter, N.; Bruce, M. Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. Research in Veterinary Science. April, 2002; 72 (Supplement A): 45. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
    NAL call no. 41.8 R312
    Descriptors: sheep, BSE, bovine spongiform encephalopathy, prion proteins, experimental infection via oral route, distribution in the body.

68. Fraser, J.R. What is the basis of transmissible spongiform encephalopathy induced neurodegeneration and can it be repaired? Neuropathology and Applied Neurobiology 2002 Feb; 28(1): 1-11. ISSN: 0305-1846.
    Abstract: Once an animal becomes infected with a prion disease, or transmissible spongiform encephalopathy (TSE), the progression of infection is relentless and inevitably fatal, although often with such prolonged incubation periods that an alternative cause of death can intervene. Infection has been compared to 'setting a clock' which then runs inexorably as the disease spreads, usually through the lymphoreticular system and then via peripheral nerves to the central nervous system (CNS), although the mechanism controlling the protracted progression is not known. Clinical disease develops as characteristic degenerative changes in the CNS progress, but the molecular basis for this pathology is not clear, particularly the relationship between the deposition of abnormal PrP and neuronal dysfunction. Recent research has identified several means of slowing (if not stopping) the clock when infection has not yet reached the CNS; although the potential for later stage therapies seems limited, neuroprotective strategies which have been shown to be effective in other neurodegenerative conditions may also ameliorate TSE induced CNS pathology. This review focuses on our current knowledge of the key events following infection of the CNS and the opportunities for intervention once the CNS has become infected.
    Descriptors: prion disease, TSE, review of current knowledge of infection, possible opportunities for intervention of disease process.

69. Frey, Jacques. Bovine spongiform encephalopathy: Are the cows mad or full of carbohydrates? Clinical Chemistry and Laboratory Medicine. February, 2002; 40 (2): 101-103.
    Descriptors: dairy cows, diet, dysregulation of carbohydrate metabolism, neurodegenerative disorders, BSE, origin, prion proteins.

70. Gargani, G. Le encefalopatie spongiformi trasmissibili. Storia, epidemiologia, ipotesi eziologiche. [Transmissible spongiform encephalopathies. History, epidemiology, etiological, hyphotheses]. Minerva Medica 2002 Feb; 93(1): 59-73. ISSN:0026-4806. In Italian.
    Abstract: The history of transmissible spongiform encephalopathies is shortly reviewed beginning with the Westminster parliament act in the year 1755 up to the description in 1996 of the variant of the Creutzfeldt-Jakob disease, transmitted from cattle to man by alimentary route. The epidemiological patterns of encephalopathies of the various animal species and of the four encephalopathies up to date reported in man are shortly described: Creutzfeldt-Jakob disease, Kuru, Gerstmann-Straussler-Scheinker disease, Fatal Familial Insomnia. Etiological hypotheses are discussed until the identification of Prions: PrPcell, on the surface of normal cells, PrPscr in the brain of humans and animals dead for these diseases. The strains of the PrPscr are described on the basis of some characters observed through the passages in rodents and of molecular pattern. The possible future epidemiological evolution of the vCJD is also discussed.
    Descriptors: TSEs, historical review, 1755-1996, NvCreutzfeldt-Jakob Disease, cattle, man, epidemiology, Creutzfeldt-Jakob disease, Kuru, Gerstmann-Straussler-Scheinker disease, Fatal Familial Insomnia, PrPscr strains.

71. Ghani, Azra C. The epidemiology of variant Creutzfeldt-Jakob disease in Europe. Microbes and Infection. 2002 Mar; 4(3): 385-93.ISSN: 1286-4579.
    Abstract: Variant Creutzfeldt-Jakob disease is one of a family of neurodegenerative diseases, first diagnosed in 1996. Scientific evidence strongly supports the hypothesis that it is acquired through consumption of bovine spongiform encephalopathy-infected meat. The majority of cases have been diagnosed in the UK in young individuals, with an excess of cases in the north and a significant cluster of cases in Leicestershire. Many uncertainties in its biology and epidemiology, in particular the length of the incubation period, make predictions of any future epidemic difficult. Studies are currently under way to obtain more precise estimates of the prevalence of asymptomatic infection through testing tonsil and appendix tissues for the abnormal prion protein.
    NAL call no. QR180 M53
    Descriptors: NvCreutzfeldt-Jakob Disease, bovine spongiform infected meat consumption, human cases, young, biology, epidemiology, incubation period, estimates of asymptomatic infection.

72. Ghani, Azra C.; Donnelly, Christl A.; Ferguson, Neil M..; Anderson, Roy M. The transmission dynamics of BSE and vCJD. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 37-47.
    DOI: 10.1016/S1631-0691(02)01389-6
    NAL call no. Q2 C6
    Descriptors: BSE, cattle, NvCreutzfeldt-Jakob disease, epidemiological models, transmission dynamics, case projections, Great Britian, Northern Ireland, Portugal, France.

73. Gray, George; Kreindel, Silvia; Ropeik, David. Mad cow disease risk in the United States. Does perceived threat overshadow true likelihood of occurrence? Postgraduate Medicine. 2002 Feb; 111(2): 13-4,16. ISSN: 0032-5481.
    Descriptors: BSE, bovine spongiform encephalopathy, animal feeds, epidemiology, transmission, NvCreutzfeldt-Jakob Disease, public health risk factors.

74. Groschup, Martin H.; Stolze, Anne. BSE- und Scrapie-Diagnostik in Deutschland. [BSE and scrapie diagnosis in Germany]. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 106-10 ISSN: 0005-9366. In German.
    Abstract: The detection of pathological prion protein is considered as pathognomonic for the diagnosis of transmissible spongiform encephalopathies. According to the EU regulations cattle older than 30 months of age (Germany 24 months) and slaughtered for human consumption must be tested by using BSE rapid tests. Likewise must be fallen stock and clinically affected animals. This article gives an overview over the diagnostic hierarchy and organization of the diagnostic system for BSE and scrapie in Germany. All suspect cases found by rapid testing are reinvestigated and clarified by the National reference laboratory for these diseases which is part of the recently founded Institute of Novel and Emerging Infectious Diseases at the Federal Research Centre for Virus Diseases of Animals located on the isle of Riems. Until the end of 2001 130 BSE cases were confirmed out of 230 submissions.
    NAL call no. 41.8 B45
    Descriptors: cattle, slaughtered animals, BSE and scrapie rapid tests, diagnostic hierarchy, Germany.

75. Gunn, H. M.; Lynch, D.; Sheridan, H.; Costelloe, A.; Weavers, E.; McElroy, M.; Cooney, J.; Sammin, D.; Good, M.; O' Sullivan, M.; Caulfield, L.; Kennedy, S.; Smyth, K.; Carr, M.; Sheridan, J.; Casey, V.; Curley, L.; Lynch, M.; Gleeson, B. Alteration in age profile of BSE cases in Ireland. Irish Veterinary Journal. 2002, 55: 2, 84-85.
    NAL call no. 41.8 IR4
    Descriptors: bovine spongiform encephalopathy, age comparison, disease incidence, Irish Republic, UK.

76. Harrison, M. The dairy industry looks to the future. Food Science and Technology. 2002, 16: 1, 24-30.

77. Heppner, F. L.; Aguzzi, A. Immunitat gegen Prionen? [Immunity against prions?] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 328-30. ISSN: 0012-0472. In German.
    NAL call no. 448.8 D48
    Descriptors: bovine spongiform encephalopathy, prions, immunology, scrapie, B and T lymphocytes, cattle, cell cultures, disease prevention and control, mice.

78. Hernandez-Sanchez, Jules; Waddington, Dave; Wiener, Pamela; Haley, Chris S; Williams, John L. Genome-wide search for markers associated with bovine spongiform encephalopathy. Mammalian Genome. 2002 Mar; 13(3): 164-8 ISSN: 0938-8990.
    Abstract: A genome-wide search for markers associated with BSE incidence was performed by using Transmission-Disequilibrium Tests (TDTs). Significant segregation distortion, i.e., unequal transmission probabilities of alleles within a locus, was found for three marker loci on Chromosomes (Chrs) 5, 10, and 20. Although TDTs are robust to false associations owing to hidden population substructures, it cannot distinguish segregation distortion caused by a true association between a marker and bovine spongiform encephalopathy (BSE) from a population-wide distortion. An interaction test and a segregation distortion analysis in half-sib controls were used to disentangle these two alternative hypotheses. None of the markers showed any significant interaction between allele transmission rates and disease status, and only the marker on Chr 10 showed a significant segregation distortion in control individuals. Nevertheless, the control group may have been a mixture of resistant and susceptible but unchallenged individuals. When new genotypes were generated in the vicinity of these three markers, evidence for an association with BSE was confirmed for the locus on Chr 5.
    NAL call no. QL738.5.M359
    Descriptors: BSE, cattle, Transmission-Disequilibrium Tests, Chromosomes 5, 10, 20, genome-wide markers

79. Herrmann, Lynn M.; Baszler, Timothy V.; Knowles, Donald P.; Cheevers, William P. PrPSc is not detected in peripheral blood leukocytes of scrapie-infected sheep: Determining the limit of sensitivity by immunohistochemistry. Clinical and Diagnostic Laboratory Immunology. March, 2002; 9 (2): 499-502.
    Descriptors: Peripheral blood leukocytes, scrapie-infected sheep, presence of PrPSc, dissociated retropharyngeal lymph node (DRLN) cells, immunohistochemistry, detection methods.

80. Hildebrandt, G.; Luy, J.; Simon, O. Kannibalismus und Rinderwahn: Ein Argument gegen jegliche Tiermehlfuetterung? [Cannibalism and mad cows: An argument against feeding of mammalian meat and bone meal (MBM)?] Tieraerztliche Umschau. 1 Februar, 2002; 57 (2): 77-89. In German.
    NAL call no. 41.8 T445
    Descriptors: bovine spongiform encephalopathy, cattle, feed formulations, mammalian meat and bone meal, cannibalism, offal recycling, rendering.

81. Hill, Andrew E.; Collinge, John Species-barrier-independent prion replication in apparently resistant species. Acta Pathologica, Microbiologica, et Immunologica Scandinavica. 2002 Jan; 110(1): 44-53. ISSN: 0903-4641.
    Abstract: Prion diseases of humans and animals are associated with the accumulation of an abnormal isoform (PrP(Sc)) of the host-encoded prion protein (PrP(C)). Transmission of these diseases between mammalian species is usually limited by a 'species barrier', which can be mediated by differences in primary sequence of the prion protein between donor and host species. Studies on species barriers usually rely on the development of clinical disease in inoculated animals as an assessment of susceptibility in a particular host. Recent studies by a number of groups have demonstrated that the absence of clinical symptoms does not necessarily exclude transmission of prion disease across a species barrier. Such results indicate that subclinical or carrier states exist in these diseases, which has public health implications regarding human exposure to BSE prions and iatrogenic transmission from apparently healthy humans. Here the issue of subclinical prion diseases is reviewed and implications are discussed.
    NAL call no. QR1.A6
    Descriptors: prion diseases, hamsters, mice, laboratory animal models, subclinical and carrier state implications for human disease, BSE, iatrogenic transmission, review.

82. Holada, Karel; Vostal, Jaroslav G.; Theisen, Patrick W.; MacAuley, Claudia; Gregori, Luisa; Rohwer, Robert G. Scrapie infectivity in hamster blood is not associated with platelets. Journal of Virology. May, 2002; 76 (9): 4649-4650. ISSN: 0022-538X.
    DOI: 10.1128/JVI.76.9.4649-4650.2002
    Abstract: The infectivity of hamster scrapie strain 263K was measured in platelets isolated from blood pooled from six hamsters with clinical scrapie. The total number of infectious doses present in the blood pool was 220, out of which only 3.5 infectious doses were associated with platelets. A larger proportion of the total infectivity was recovered from the mononuclear leukocyte fraction. This result indicates that platelets are not the source of blood-borne infectivity in transmissible spongiform encephalopathy-infected hamsters.
    NAL call no. QR360.J6
    Descriptors: scrapie strain 263K, infected hamster blood, platelet fraction analysed, mononuclear leukocytes, infective source, scrapie transmission.

83. Hooper, Nigel M. Prion disease: Close encounters of the cellular kind. Current Biology. April 2, 2002; 12 (7): R248-R249.
    DOI: 10.1016/S0960-9822(02)00783-2
    NAL call no. QH301.C85
    Descriptors: prion diseases, BSE, CJD.

84. Horby, Peter. Rendering beef safe. Clinical Infectious Diseases. 1 January, 2002; 34 (1): 129. ISSN: 1058-4838.
    NAL call no. RC111 R4
    Descriptors: food products, beef, human health risks, BSE.

85. Hsich, Gary; Kenney, Kimbra; Gibbs, Clarence J.Jr; Harrington, Michael, G. Method of detecting transmissible spongiform encephalopathies. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] June 18, 2002; 1259 (3): No Pagination.
    Journal URL: www.uspto.gov/web/menu/patdata.html
    NAL call no. T223 A22
    Descriptors: detection method, 14-3-3 proteins, cerebrospinal fluid, elevated levels indicate transmissible spongiform encephalopathies, CJD in humans, BSE in cattle.

86. Huang, Fang Ping; Farquhar, Christine F.; Mabbott, Neil A.; Bruce, Moira E.; MacPherson, G. Gordon. Migrating intestinal dendritic cells transport PrP(Sc) from the gut. Journal of General Virology. 2002 Jan; 83(Pt 1): 267-71. ISSN: 0022-1317.
    Abstract: Bovine spongiform encephalopathy, variant Creutzfeldt-Jakob disease (vCJD) and possibly also sheep scrapie are orally acquired transmissible spongiform encephalopathies (TSEs). TSE agents usually replicate in lymphoid tissues before they spread into the central nervous system. In mouse TSE models PrP(c)-expressing follicular dendritic cells (FDCs) resident in lymphoid germinal centres are essential for replication, and in their absence neuroinvasion is impaired. Disease-associated forms of PrP (PrP(Sc)), a biochemical marker for TSE infection, also accumulate on FDCs in the lymphoid tissues of patients with vCJD and sheep with natural scrapie. TSE transport mechanisms between gut lumen and germinal centres are unknown. Migratory bone marrow-derived dendritic cells (DCs), entering the intestinal wall from blood, sample antigens from the gut lumen and carry them to mesenteric lymph nodes. Here we show that DCs acquire PrP(Sc) in vitro, and transport intestinally administered PrP(Sc) directly into lymphoid tissues in vivo. These studies suggest that DCs are a cellular bridge between the gut lumen and the lymphoid TSE replicative machinery.
    NAL call no. QR360.A1J6
    Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob disease, vCJD, sheep scrapie, orally acquired transmissible spongiform encephalopathies, TSEs, mouse models, transport of dendritic cells directlyinto lymphoid tissues in vivo, replicatation.

87. Ingrosso, Loredana; Vetrugno, Vito; Cardone, Franco; Pocchiari, Maurizio Molecular diagnostics of transmissible spongiform encephalopathies. Trends in Molecular Medicine. June, 2002; 8 (6): 273-280.
    Descriptors: post mortem diagnosis, immunochemical based kits, PrPSc, preclinical screening tests, blood, blood derived products, meat safety, Europe.

88. Ironside, James W. Neuropathology of variant Creutzfeldt-Jakob disease. [Neuropathologie de la maladie variant de Creutzfeldt Jakob]. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 27-31.
    DOI: 10.1016/S1631-0691(02)01381-1
    NAL call no. Q2 C6
    Descriptors: human prion diseases, NvCreutzfeldt-Jakob Disease, BSE, florid cluster plaques, cerebrum, cerebellum, PrPRES, morphology, histology, biochemistry, codon 129, compared to CJD.

89. Ironside, J.W.; McCardle, L.; Horsburgh, A.; Lim, Z.; Head, M.W. Pathological diagnosis of variant Creutzfeldt-Jakob disease. APMIS. Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 79-87 ISSN: 0903-4641.
    Abstract: The neuropathological and biochemical features of the 89 histologically confirmed cases of variant Creutzfeldt-Jakob disease (vCJD) diagnosed up to the end of October 2001 in the UK are reviewed. Histology of the central nervous system, lymphoid tissues and other organs was accompanied by immunocytochemistry and Western blot analysis of the disease-associated form of the prion protein (PrP(RES)). All patients with vCJD were methionine homozygotes at codon 129 of the PrP gene. The pathology of vCJD showed relatively uniform morphological and immunocytochemical characteristics, which were distinct from other forms of CJD. PrP(RES) accumulation was widespread in lymphoid tissues in vCJD, but was not identified in other non-neural tissues. PrP(RES) in vCJD brain tissue showed a uniform glycotype pattern distinct from sporadic CJD. Given the increasingly widespread occurrence of bovine spongiform encephalopathy in Europe and Asia, there is a major need for widespread CJD surveillance. This should be accompanied by a multidisciplinary laboratory approach to the investigation and diagnosis of all forms of CJD, with the need to investigate autopsy tissues from suspected cases by the histological and biochemical techniques described herein.
    NAL call no. QR1.A6
    Descriptors: NvCreutzfeldt-Jakob Disease, histology, histology, immunocytochemistry, Western blot analysis, CNS, lymphoid tissue, other organs.

90. Kaaden, O.R.; Eichhorn, W.; Essbauer, S. Recent developments in the epidemiology of virus diseases. J Vet Med, Ser B. Berlin : Blackwell Wissenschafts-Verlag. Feb 2002. v. 49 (1) p. 3-6. ISSN: 0931-1793.
    Abstract: There is continual variation in viral epidemics regarding clinical symptoms, duration and disappearance, and the emergence of new diseases. This can be observed in both human and animal diseases. This evolution of virus diseases is mainly related to three factors: aetiological agent, host and environment. As far as genetic alterations of the virus are concerned, two major mechanisms are involved: mutations such as recombination and reassortment; and selection for resistance or susceptibility. This review focuses on the epidemiology of newly emerged virus diseases in man and animals, such as acquired immunodeficiency syndrome, haemorraghic fevers, bovine spongiform encephalopathy, canine haemorraghic disease and respiratory syndrome in horses.
    NAL call no. 41.8 Z52
    Descriptors: human, livestock, viral diseases, epidemiology, epidemics, symptoms, duration, evolution, etiology, genetic variation, mutations, recombination, natural selection, hemorrhagic fevers, bovinespongiform encephalopathy, literature reviews, acquired immune deficiency syndrome, canine hemorrhagic disease, respiratory syndrome in horses.

91. Kahler, Susan C. The rationale for ridding U.S. of scrapie. Journal of the American Veterinary Medical Association. 2002 May 1; 220(9): 1280-1. ISSN: 0003-1488
    NAL call no. 41.8 AM3
    Descriptors: BSE, bovine spongiform encephalopathy, prevention and control, scrapie, cattle, sheep, animal feed formations and processing, contaminated feeds, prion diseases, US.

92. Kao, R. R.; Gravenor M.B.; Baylis, M.; Bostock, C.J.; Chihota, C. M.; Evans, J.C.; Goldmann, W.; Smith, A. J. A.; McLean, A.R. The potential size and duration of an epidemic of bovine spongiform encephalopathy in British sheep. Science. Washington, DC. 11 January, 2002; 295 (5553): 332-335. ISSN: 0036-8075
    Abstract: Because there is a theoretical possibility that the British national sheep flock is infected with bovine spongiform encephalopathy (BSE), we examined the extent of a putative epidemic. An age cohort analysis based on numbers of infected cattle, dose responses of cattle and sheep to BSE, levels of exposure to infected feed, and number of BSE-susceptible sheep in the United Kingdom showed that at the putative epidemic peak in 1990, the number of cases of BSE-infected sheep would have ranged from fewer than 10 to about 1500. The model predicts that fewer than 20 clinical cases of BSE in sheep would be expected in 2001 if maternal transmission occurred at a rate of 10%. Although there are large uncertainties in the parameter estimates, all indications are that current prevalence is low; however, a simple model of flock-to-flock BSE transmission shows that horizontal transmission, if it has occurred, could eventually cause a large epidemic.
    NAL call no. 470 Sci2
    Descriptors: BSE, bovine spongiform encephalopathy, cattle, sheep diseases, horizontal disease transmission, cohort studies, genetic predisposition to Disease, Great-Britain, epidemiology, logistic models, prevalence, prions chemistry and genetics, probability, scrapie epidemiology and transmission, sheep genetics.

93. Kaup F. J.; Schwibbe, M. Primaten als Versuchstiere. [Nonhuman primates as laboratory animals.] DTW Deutsche-Tieraerztliche-Wochenschrift. Marz, 2002; 109 (3): 104-108. In German.
    NAL call no. 41.8 D482
    Descriptors: non-human primates, biomedical models, disease models, viral infections, transmissible spongiform encephalopathies.

94. Kersseboom, R.; Koekoek, S. C.; Richardus, J. H. Het risico van de variant van de ziekte van Creutzfeldt-Jakob in Nederland en het effect van preventieve maatregelen. [The risk of variant Creutzfeldt-Jakob disease in the Netherlands and the effect of preventive measures]. Nederlands Tijdschrift voor Geneeskunde. 2002 Apr 20; 146(16): 754-9 ISSN: 0028-2162. In Dutch.
    Abstract: Variant Creutzfeldt-Jakob disease (vCJD) is a fatal and untreatable neurological disease, in which pathogenic prions (PrPSc) are involved. There is convincing epidemiological and experimental evidence that vCJD is a human expression of bovine spongiform encephalopathy (BSE). The risk of transmission of pathogenic prions which cause vCJD to humans is influenced by the species barrier, genetic susceptibility of the host, dose of infection and route of exposure. Transmission of pathogenic prions from bovines to humans is possible through meat products containing nerve and lymphatic tissue, and through medical products derived from bovine material. Human to human transmission is, in principle, also possible via blood and blood-derived products, human organs and tissues for transplantation, and through surgical instruments. Preventive measures to reduce transmission from bovines to humans have been introduced step by step in the Netherlands since 1989. With proper implementation, the current risk of becoming infected by Dutch meat products is small. It is very likely, however, that in the past decade BSE-infected bovines have entered the food chain. The Dutch population has also been exposed to foreign infected meat products. Measures to prevent human to human transmission are currently being improved in the Netherlands. It is expected that cases of vCJD will occur in the Netherlands in the future, but the number cannot be estimated accurately. The presence of PrPSc in both the livestock and in the human population in the Netherlands constitutes a permanent public health threat and is a reason for continued vigilance and active prevention.
    Descriptors: NvCJD, transmission of pathogenic prions, control measures, public health concerns, Netherlands, risk assessment, level of PrPSc in livestock and people.

95. Kirk, Sara F. L.; Greenwood, Darren; Cade, Janet E.; Pearman, Alan D. Public perception of a range of potential food risks in the United Kingdom. Appetite. June, 2002; 38 (3): 189-197. ISSN: 0195-6663
    DOI: 10.1006/appe.2001.0478
    NAL call no. QP141.A1A64
    Descriptors: public/consumer survey, potential food risks, BSE, bovine spongiform encephalopathy, Salmonella, 1998, 1999, UK.

96. Kneipp, Janina; Beekes, Michael; Lasch, Peter; Naumann, Dieter. Molecular changes of preclinical scrapie can be detected by infrared spectroscopy. Journal of Neuroscience. April 15, 2002; 22 (8): 2989-2997.
    URL: http://www.jneurosci.org/cgi/content/abstract/22/8/2989
    Descriptors: infrared microspectroscopy, molecular changes, cryosections, scrapie-infected brain tissue, 263K scrapie infected hamster nervous tissue, medulla oblongata.

97. Krebs, John R.; May, Robert M.; Stumpf, Michael P.H. Theoretical models of sheep BSE reveal possibilities. Nature. 2002 Jan 10; 415(6868): 115 ISSN: 0028-0836.
    DOI: 10.1038/415115a
    NAL call no. 472 N21
    Descriptors: bovine spongiform encephalopathy, etiology, sheep diseases, animal feed formulations, cattle, disease prevention and control, public policy, risk assessment.

98. Kohnlein, C.; Poser, S. Schwerpunktheft Creutzfeldt-Jakob-Krankheit/BSE. [Creutzfeldt-Jakob disease/BSE special issue]. Deutsche Medizinische Wochenschrift. 2002 Jun 14; 127(24): 1344. ISSN: 0012-0472. In German.
    NAL call no. 448.8 D48
    Descriptors: BSE, humans, cattle, epidemiology, historical notes, risk factors, transmission, UK, Scotland.

99. Larski, Zdzislaw Niektore nowe dane dotyczace wirusologii i zakaznych gabczastych encefalopatii. [Some new data concerning virology and transmittable spongiform encephalopathies.] Medycyna-Weterynaryjna. 2002; 58 (1): 13-17. In Polish.
    NAL call no. 41.8 M463
    Descriptors: review, preclinical diagnosis, transmissible spongiform encephalopathies, TSE.

100. Laude, Hubert; Vilette, Didier; Le Dur, Annick; Archer, Fabienne; Soulier, Solange; Besnard, Nathalie; Essalmani, Rachid; Vilotte, Jean-Luc New in vivo and ex vivo models for the experimental study of sheep scrapie: Development and perspectives. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 49-57.
     DOI: 10.1016/S1631-0691(02)01393-8
     NAL call no. Q2 C6
     Descriptors: sheep, scrapie, transmissible spongiform encephalopathy, TSE, transgenic mouse models-tgOv, invitro cell lines expressing ovine prion proteins, rabbit epithelial cell line.

101. Lawlor, D.; Hockley, J.; Hawkins, S.A.C.; Simmons, M.M.; Matthews, D. The TSE Archive at VLA Weybridge. Research in Veterinary Science. April, 2002; 72 (Supplement A): 46. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: transmissible spongiform encephalopathies, prions.

102. Lehmann, Sylvain. Metal ions and prion diseases. Current Opinion in Chemical Biology. April, 2002; 6 (2): 187-192.
     Descriptors: prion, metal ions, BSE, other prion diseases.

103. Lehmann, Sylvain; Beranger, Florence; Solassol, Jerome; Ceschia, Audrey; Perrier, Veronique; De Gassart, Aude; Vilette, Didier; Laude, Hubert; Kellermann, Odile; Mange, Alain. Modeles en culture cellulaire des encephalopathies spongiformes transmissibles. [Cell culture models of transmissible spongiform encephalopathies.] Comptes Rendus Biologies. Janvier, 2002; 325 (1): 59-65.
     DOI: 10.1016/S1631-0691(02)01391-4
     NAL call no. Q2 C6
     Descriptors: invitro cell cultures, experimental models, prion infected lines, wild-type, mutated or chimeric prion proteins, useful for investigating the biology of pathogenicity.

104. Levieux, A.; Rivera, V.; Levieux, D. Immunochemical control of the species origin of porcine crude heparin and detection of ovine and caprine materials. Journal of Pharmaceutical and Biomedical Analysis. 2002 Jan 1. 27(1-2): 305-313 ISSN: 0731-7085
     Abstract: As a consequence of the outbreak of bovine spongiform encephalopathy (BSE), ruminants materials have been generally banned from the production of heparin. Immunochemical methods have been recently developed for the control of the raw materials used by manufacturers of materials such as porcine mucosa and for the detection of bovine crude heparins. To certify the porcine origin of crude porcine heparins and to exclude ovine or caprine materials, new ELISAs were developed. Rabbit antisera were produced against species-specific antigenic contaminants present in crude heparins or in eluted materials (EM) from the chromatographic step of the purification process. When analysed by line immunoelectrophoresis, these antisera revealed five to eleven antigenic contaminants in the EMs, the major one being the most anodic and predominant antigen in crude heparins. Using the best antisera, competitive indirect ELISAs were optimised. They allowed the detection of porcine, ovine and caprine crude heparins down to a dilution of 0.6 to 1.5 parts per 1000, with CVs ranging from 3 to 12%. These ELISAs complete the set of immunological techniques which can be routinely used by heparin manufacturers to secure their supply chain.
     Descriptors: BSE, bovine based pharmaceuticals, health risks, ELISA test, antibodies.

105. Lucker, Ernst; Hardt, Michael; Groschup, Martin H. Detection of CNS and PrPSc in meat products. Berliner und Munchener Tierarztliche Wochenschrift 2002 Mar-Apr; 115(3-4): 111-7. ISSN: 0005-9366.
     Abstract: Several methods for the detection of tissues of the central nervous system (CNS) in meat products have been developed and partly validated for use in official food control as pertaining to human BSE-exposure risk. So far, however, methods for the detection of abnormal prion protein (PrPSc) were not evaluated for their potential applicability to the matrix of heat treated meat products. We developed a micro technological procedure for the preparation of meat products suitable for high security laboratories as masses were 6 to 8 orders of magnitude lower than in conventional meat technology. Thus it was possible to produce standard micro sausages containing defined amounts of bovine BSE-positive brain. This material showed all characteristics of normal meat products and a homogeneous distribution of brain as indicated by NSE and GFAP western immunoblotting and GFAP immunometric analyses. Using a commercially available and certified immunometric assay for detection of PrPSc in untreated brain it was possible to detect BSE-positive CNS down to a content of 0.25% in heat treated meat products. We found a high correlation between PrPSc OD-values and CNS content and linearity up to 10% CNS. In 30 samples of retail meat products no sample transgressed the official cut off value for untreated bovine brain. Further studies are needed to show whether an increase of sensitivity in PrPSc detection from the meat product matrix is possible, in particular by optimisation of the extraction procedure.
     NAL call no. 41.8 B45
     Descriptors: CNS, PrPSc, detection of central nervous tissue in meat, immunometric assay, detection of PrPSc in untreated brain.

106. Lucker, Ernst; Schlottermuller, Beate; Martin, Antje Studies on contamination of beef with tissues of the central nervous system (CNS) as pertaining to slaughtering technology and human BSE-exposure risk. Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 118-21 ISSN: 0005-9366
     Abstract: Contamination of beef by tissues of the central nervous system (CNS) due to slaughter technology causes some concern considering the potential health hazard by food borne exposure to the infectious agent of BSE. The present study was designed to quantify the extent of CNS contamination as pertaining to stunning and splitting technology. Of the 726 animals 48 contained a total of 58 emboli-like particles in lungs and/or right ventricles. The incidence of emboli-like particles was found to be slightly higher in animals slaughtered without pithing (5.9%) than in the animals slaughtered with pithing (4.1%). Of the 58 emboli-like particles only two were positive in the anti-NSE western immunoblotting (0.3% of the 726 animals). The immuno reaction of these NSE-positive particles was several orders of magnitude lower as obtained by pure brain material. The microscopical analysis of the two NSE-positive emboli-like particles for presence of CNS-like tissues was negative. Following splitting of carcasses by sawing with and without prior removing the spinal cord we found NSE-positive reactions in 32% and 17% of the samples, respectively. The immuno reaction, however, was predominantly comparable to standard material containing less than 0.5% CNS. Overall the results show that CNS contamination of bovine carcasses cannot be excluded by current slaughter technology. However, the additional human BSE-exposure risk can be judged to be at least minor when considering extent of contamination, dilution effects and BSE-testing.
     NAL call no. 41.8 B45
     Descriptors: cattle, slaughter processes, level of beef meat contamination, central nervous system tissue, emboli-like particles.

107. Lunney, Joan K.; Fossum, Caroline; Alm, Gunnar V.; Steinbach, Falko; Wattrang, Eva. Veterinary immunology: Opportunities and challenges. Trends in Immunology. January, 2002; 23 (1): 4-6. 6th International Veterinary Immunology Symposium, Uppsala, Sweden, July 15-20, 2001. ISSN: 1471-4906
     NAL call no. QR180 I56
     Descriptors: Veterinary medicine, animal immunology.

108. Lupi, Omar. Prions in dermatology. Journal of the American Academy of Dermatology. 2002 May; 46(5): 790-3 ISSN: 0190-9622
     Abstract: Prion diseases are uncommon fatal neurodegenerative disorders that have gained scientific importance as a result of the emergence of new forms of these diseases in both animals and humans. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein. There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy ("mad cow disease") has affected humans. Recent studies have demonstrated that prions can adhere easily to metal surfaces, and normal sterilization procedures are not likely to completely inactivate them. Iatrogenic transmission of prion diseases, such as Creutzfeldt-Jakob disease, was recognized after corneal transplantations, dura mater grafts, neurosurgical procedures, and the use of human hormones (growth hormone and gonadotropin). Although bovine collagen has long been recognized as a safe and biocompatible material, dermatologists should be aware of the theoretical potential for prion transmission when materials from bovine origin and products obtained from cultured cells fed with fetal or newborn calf serum are used.
     Descriptors: spongiform encephalopathies, prions, iatrogenic transmission, bovine based products, fetal or newborn calf serum, risks.

109. Mabbott, Neil A; McGovern, Gillian; Jeffrey, Martin; Bruce, Moira E. Temporary blockade of the tumor necrosis factor receptor signaling pathway impedes the spread of scrapie to the brain. Journal of Virology 2002 May; 76(10): 5131-9. ISSN: 0022-538X
     DOI: 10.1128/JVI.76.10.5131-5139.2002
     Abstract: Although the transmissible spongiform encephalopathies (TSEs) are neurodegenerative diseases, their agents usually replicate and accumulate in lymphoid tissues long before infection spreads to the central nervous system (CNS). Studies of a mouse scrapie model have shown that mature follicular dendritic cells (FDCs), which express the host prion protein (PrP(c)), are critical for replication of infection in lymphoid tissues. In the absence of mature FDCs, the spread of infection to the CNS is significantly impaired. Tumor necrosis factor alpha (TNF-alpha) secretion by lymphocytes is important for maintaining FDC networks, and signaling is mediated through TNF receptor 1 (TNFR-1) expressed on FDCs and/or their precursors. A treatment that blocks TNFR signaling leads to the temporary dedifferentiation of mature FDCs, raising the hypothesis that a similar treatment would significantly delay the peripheral pathogenesis of scrapie. Here, specific neutralization of the TNFR signaling pathway was achieved through treatment with a fusion protein consisting of two soluble human TNFR (huTNFR) (p80) domains linked to the Fc portion of human immunoglobulin G1 (huTNFR:Fc). A single treatment of mice with huTNFR:Fc before or shortly after intraperitoneal injection with the ME7 scrapie strain significantly delayed the onset of disease in the CNS and reduced the early accumulation of disease-specific PrP in the spleen. These effects coincided with a temporary dedifferentiation of mature FDCs within 5 days of huTNFR:Fc treatment. We conclude that treatments that specifically inhibit the TNFR signaling pathway may present an opportunity for early intervention in peripherally transmitted TSEs.
     NAL call no. QR360.J6
     Descriptors: scrapie, TSE's mouse scrapie model, follicular dendritic cells, replication of i nfection in lymphoid tissues, blocking tumor necrosis factor alpha, pathogenesis delays.

110. MacDiarmid, S.C. Bovine spongiform encephalopathy (BSE) in sheep? Australian Veterinary Journal 2002 Mar; 80(3): 148-9 ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: BSE, epidemiology, disease transmission, cattle, goats, sheep, population surveillance methods, risk assessment, etiology, Australia, New Zealand.

111. Marella, Mathieu; Lehmann, Sylvain; Grassi, Jacques; Chabry, Joelle. Filipin prevents pathological prion protein accumulation by reducing endocytosis and inducing cellular PrP release. Journal of Biological Chemistry, 2002 Jul 12; 277(28): 25457-64. ISSN: 0021-9258
     Abstract: Conversion of the normal membrane-bound prion protein (PrP-sen) to its pathological isoform (PrP-res) is a key event in the pathogenesis of transmissible spongiform encephalopathies. Although the subcellular sites of conversion are poorly characterized, several lines of evidence have suggested the involvement of membrane lipid rafts in the conversion process. Here we report that copper stimulates the endocytosis of PrP-sen via a caveolin-dependent pathway in both microglia and neuroblastoma cells. We show that the polyene antibiotic filipin both limits endocytosis of PrP-sen and dramatically reduces the amount of membrane-bound PrP-sen. This reduction results from a rapid and massive release of full matured PrP-sen into the culture medium. Finally, we demonstrate that filipin is a potent inhibitor of PrP-res formation into chronically infected neuroblastoma cells. Our results reinforce the role of rafts in PrP trafficking and raise the possibility that the release of PrP-sen from the plasma membrane decreases the amount of available substrate PrP-sen at the conversion sites.
     NAL call no. 381 J824
     Descriptors: cell culture, microglia, neuroblastoma cells, copper, endocytosis, Prp-sen, effects of filipin antibiotic, inhibition of PrP-res formation, nystatin pharmacology.

112. Martin, S.; Gonzalez, L.; Jeffrey, M.; Bellworthy, S.J. Differential diagnosis of BSE agent and scrapie infection of sheep. Research in Veterinary Science. April, 2002; 72 (Supplement A): 44. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: bovine spongiform encephalopathy, scrapie, diagnostic techniques.

113. Martinsen, T.C.; Taylor, D.M.; Johnsen, R.; Waldum, H.L. Gastric acidity protects mice against prion infection? Scandinavian Journal of Gastroenterology. May, 2002; 37 (5): 497-500.
     URL: http://www.ingentaconnect.com/content/apl/sgas/2002/00000037/00000005/art00001
     NAL call no. RC799 S33
     Descriptors: transmissible degenerative encephalopathies, sheep scrapie, bovine spongiform encephalopathy, Creutzfeldt-Jakob Disease, gastric acidity and susceptibility to infection, mouse model, experimentalinfection, implications for eating contaminated meats.

114. Matorras, Roberto; Rodriguez Escudero, Francisco, J. The use of urinary gonadotrophins should be discouraged. Human Reproduction. Oxford. July, 2002; 17 (7): 1675.
     NAL call no. QP251 H85
     Descriptors: prion proteins, infectivity concerns, transmissible spongiform encephalopathies, FSH, LH.

115. McCormack, James E.; Baybutt, Herbert N.; Everington, Dawn; Will, Robert G.; Ironside, James W.; Manson, Jean C. PRNP contains both intronic and upstream regulatory regions that may influence susceptibility to Creutzfeldt-Jakob Disease. Gene. 2002 Apr 17; 288(1-2): 139-46 ISSN: 0378-1119
     Abstract: The Prion protein (PrP) plays a central role in Creutzfeldt-Jakob Disease (CJD) and other transmissible spongiform encephalopathies (TSEs). Mutations in the protein coding region of the human PrP gene (PRNP), which have been proposed to alter the stability of the PrP protein, have been linked to a number of forms of TSE. However, the majority of CJD cases are not associated with mutations in the PRNP coding region and alternative mechanisms must therefore underlie susceptibility to these forms of CJD. Transgenic mice, that over- or under-express PrP genes, have shown a correlation between the level of PrP gene expression and the incubation time of disease. Polymorphisms that lead to alterations in human PRNP gene expression, could therefore be candidates for influencing susceptibility of an individual to CJD. In order to investigate this hypothesis, we have defined an upstream and intronic regulatory region of the PRNP gene. Sequencing of these regions in controls, sporadic CJD (sCJD) and variant CJD (vCJD) patients has identified three polymorphisms, all of which are more common in sCJD patients than controls. Our data suggests that polymorphisms in the regulatory region of the PRNP gene may be a risk factor for CJD.
     NAL call no. QH442.A1G4
     Descriptors: risk factors for disease, humans, polymorphisms, regulatory regions.

116. Milhavet, Ollivier; Lehmann, Sylvain. Oxidative stress and the prion protein in transmissible spongiform encephalopathies. Brain Research. Brain Research Reviews. 2002 Feb; 38(3): 328-39 ISSN: 0165-0173
     Abstract: Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. These diseases are believed to be the consequence of the conformational conversion of the prion protein into an abnormal isoform. Their exact pathogenic mechanism remains uncertain, but it is believed that oxidative stress plays a central role. In this article, we will first review in detail the data supporting the latter hypothesis. Subsequently, we will discuss the relationship between the prion protein and the cellular response to oxidative stress, attempting ultimately to link PrP function and neurodegeneration in these disorders.
     Descriptors: TSE, prion protein conformation, oxidative stress, pathogenic mechanism.

117. Morgenthaler, Jean Jacques; Maring, Jacques Andre; Rentsch,Markus. Method for the removal of causative agent(s) of transmissible spongiform encephalopathies from protein solutions. Official Gazette of the United States Patent and Trademark Office Patents. [e-file] June 18, 2002; 1259 (3): No Pagination
     Journal URL: www.uspto.gov/web/menu/patdata.html
     NAL call no. T223 A22
     Descriptors: non-conventional transmissible agents, causative agents, transmissible spongiform encephalopathies, contaminated protein solution, absorbent, kieselguhr, diatomaceous earth, silicic acid, clay minerals, metal hydroxide, metal oxihydrate, cellulose, perlite, bentonite, and water-insoluble synthetic polymers.

118. Newgard, Jason R.; Rouse, Glenda C.; McVicker, Jerry K. Novel method for detecting bovine immunoglobulin G in dried porcine plasma as an indicator of bovine plasma contamination. Journal of Agricultural and Food Chemistry 2002 May 22; 50(11): 3094-7. ISSN: 0021-8561
     Abstract: Current U.S. Food and Drug Administration regulationsprohibit feeding of protein derived from mammalian tissue, excluding blood and blood products and any product that consists entirely of porcine or equine protein. A novel lateral flow immunoassay device has been developed that can quickly and qualitatively determine the presence of bovine immunoglobulin G (IgG), a major component in blood products, at very low levels (0.01% v/v). The device can be used to test for bovine IgG commingling in spray-dried porcine plasma used in the feed industry. Producers and consumers alike could use this device to verify product content at threshold levels.
     NAL call no. 381 J8223
     Descriptors: US FDA, animal feeds, and swine or horse protein comtaminants, immunoassay for bovine IgG contaminants, BSE control and prevention.

119. Nair, Bindu; Elmore, Amy R. Final report on the safety assessment of Human Placental Protein, Hydrolyzed Human Placental Protein, Human Placental Enzymes, Human Placental Lipids, Human Umbilical Extract, Placental Protein, Hydrolyzed Placental Protein, Placental Enzymes, Placental Lipids, and Umbilical Extract. International Journal of Toxicology. 2002; 21 (Supplement 1): 81-91.
     NAL call no. RA1190 J61
     Descriptors: cosmetic ingredients, human and animal pracental proteins, transmissible spongiform encephalopathies risks, EU prohibited use, FDA, toxicology recommendations.

120. Narang, Harash. A critical review of the nature of the spongiform encephalopathy agent: protein theory versus virus theory. Experimental Biology and Medicine. 2002 Jan; 227(1): 4-19. ISSN: 1535-3702.
     Abstract: All spongiform encephalopathies (SEs) result in brain disorders brought about by a slow virus. Since the origin of bovine SE (BSE), the infectious nature of the disease has been firmly established. Tubulofilamentous particles/scrapie termed nemavirus (NVP) and scrapie-associated fibrils (SAF) are ultrastructural markers, whereas protease-resistant protein (PrP(sc)) is a protein marker. The PrP molecules aggregate to form SAF. Each NVP consists of three layers: an outer protein coat, an intermediate ssDNA layer, and inner PrP/SAF. Therefore, ssDNA and PrP/SAF are physically associated with each other. The existence of at least 20 stable strains of SEs implies that a nucleic acid molecule serves as the information molecule. Animals inoculated with PrP(sc) do not develop the clinical disease, however, ssDNA purified from scrapie-hamster brains by alkaline gel electrophoresis mixed with binding proteins before inoculation developed the clinical disease. It appears that an "accessory protein" coded by the ssDNA of the NVP interacts with normal PrP(c) molecules, resulting in their conversion to PrP(sc)/SAF. The pathogenesis process in the infected animal, with increasing incubation periods, reveals that larger amounts of normal PrP molecules are modified to form SAF. This interferes with the normal supply of PrP to cell membranes, which become disrupted and eventually fragment, resulting in the vacuoles typical of those found in the SEs. Critical review of scientific literature has demonstrated that the agent contains a DNA genome.
     NAL call no. QH301 E9
     Descriptors: bovine spongiform encephalopathy, brain disorders, scrapie, PrPSc, interaction between nemavirus and scrapie associated fibrils cause the disease, pathogenesis.

121. Nunnally, Brian K. It's a mad, mad, mad, mad cow: A review of analytical methodology for detecting BSE/TSE. Trends in Analytical Chemistry. February, 2002; 21 (2): 82-89.
     DOI: 10.1016/S0165-9936(01)00134-0
     NAL call no. QD71T7
     Descriptors: review, analytical detection, bovine spongioform encephalopathy, BSE, transmissible spongioform encephalopathy, TSE, golden Syrian hamster bioassay, immunoassays and capillary electrophoresis methods, blood, bio-fluids.

122. Ockerman, H. W.; Basu, L. Update on Bovine Spongiform Encephalopathy (BSE, mad cow disease). Special Circular Ohio Agricultural-Research and Development Center. 2002, No.183, 61-65; 5 ref.
     NAL call no. 100 Oh3S
     Descriptors: BSE, state of the U.S. cattle industry.

123. Oldstone, Michael B.A.; Race, Richard; Thomas, Diane; Lewicki,Hanna; Homann, Dirk; Smelt, Sara; Holz, Andreas; Koni, Pandelakis; Lo, David; Chesebro, Bruce; Flavell, Richard. Lymphotoxin-alpha- and lymphotoxin-beta-deficient mice differ in susceptibility to scrapie: evidence against dendritic cell involvement in neuroinvasion. Journal of Virology 2002 May; 76(9): 4357-63. ISSN: 0022-538X.
     Abstract: Transmissible spongiform encephalopathy or prion diseases are fatal neurodegenerative disorders of humans and animals often initiated by oral intake of an infectious agent. Current evidence suggests that infection occurs initially in the lymphoid tissues and subsequently in the central nervous system (CNS). The identity of infected lymphoid cells remains controversial, but recent studies point to the involvement of both follicular dendritic cells (centers is dependent on lymphotoxin alpha (LT-alpha) and LT-beta signaling components. We report here that by the oral route, LT-alpha -/- mice developed scrapie while LT-beta -/- mice did not. Furthermore, LT-alpha -/- mice had a higher incidence and shorter incubation period for developing disease following inoculation than did LT-beta -/- mice. Transplantation of lymphoid tissues from LT-beta -/- mice, which have cervical and mesenteric lymph nodes, into LT-alpha -/- mice, which do not, did not alter the incidence of CNS scrapie. In other studies, a virus that is tropic for and alters functions of CD11c(+) cells did not alter the kinetics of neuroinvasion of scrapie. Our results suggest that neither FDC nor CD11c(+) cells are essential for neuroinvasion after high doses of RML scrapie. Further, it is possible that an as yet unidentified cell found more abundantly in LT-alpha -/- than in LT-beta -/- mice may assist in the amplification of scrapie infection in the periphery and favor susceptibility to CNS disease following peripheral routes of infection. FDC) and CD11c(+) lymphoid dendritic cells. FDC generation and maintenance in general.
     NAL call no. QR360 J6
     Descriptors: scrapie, oral intake, infectious material, lymphoid tissue, NCS, LT alpha mice, LT beta mice, incubation periods.

124. Paisley, Larry G. Monitoring and analysis of bovine spongiform encephalopathy (BSE) testing in Denmark using statistical models. APMIS. January, 2002; 110 (1): 61-70.
     Abstract: The evolution of monitoring and surveillance for bovine spongiform encephalopathy (BSE) from the phase of passive surveillance that began in the United Kingdom in 1988 until the present is described. Currently, surveillance for BSE in Europe consists of mass testing of cattle slaughtered for human consumption and cattle from certain groups considered to be at higher risk of having clinical or detectable BSE. The results of the ongoing BSE testing in Denmark have been analyzed using two statistical approaches: the "classical" frequentist and the Bayesian that is widely used in quantitative risk analysis. The analyses were intended to provide information for decision-markers, the media and the public as well as to provide inputs for future BSE surveillance models. The results to date suggest that the total number of BSE cases that will be found in Denmark in 2001 will not exceed 16.
     NAL call no. QR1.A6
     Descriptors: cattle, prions, bovine spongiform encephalopathy, disease surveillance, statistical methods, epidemiology, Denmark.

125. Parnham, D.W.; Foster, J. D.; Hunter, N. Immunocytochemical tales of the unexpected and the power of the PET Blot. Research in Veterinary-Science. April, 2002; 72 (Supplement A): 47-48. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002
     NAL call no. 41.8 R312
     Descriptors: prion diseases, diagnostic measures.

126. Pedersen, Nils Strandberg; Smith, Else. Prion diseases: epidemiology in man. APMIS Acta Pathologica, Microbiologica, et Immunologica Scandinavica 2002 Jan; 110(1): 14-22 ISSN: 0903-4641.
     Abstract: Prion disease in man was first described as Creutzfeldt-Jacob disease (CJD) in the 1920s. CJD may have three different origins: sporadic, familial, due to mutations in the prion gene, or infectious, due to iatrogenic exposure to infectious brain material. As an example of the latter, kuru, in Papua New Guinea, was a variant of CJD transmitted by cannibalism. Between 1957 and 1982 more than 2500 died of kuru. Sporadic CJD is the most common form of CJD and occurs with an incidence of around one per million in most parts of the world. Familial CJD accounts for approximately 10% of all European cases of CJD, and is associated with inherited mutations of the prion protein gene, caused by one of the 24 single amino acid substitutions or insertions of octapeptide repeats. CJD caused by infections involves either iatrogenic cases of CJD, resulting from exposure to infectious brain, pituitary or ocular tissue, or from ingestion of infected food items. As of today, a few hundred iatrogenic cases of CJD have been diagnosed worldwide, the majority due to transmission by cadaveric pituitary HCG. So far, 111 cases of vCJD have been diagnosed caused by BSE-contaminated food. The size of the epidemic is still unclear and worst-case scenarios indicate that we may expect many thousands of cases in the future.
     NAL call no. QR1.A6
     Descriptors: Creutzfeldt-Jacob disease, CJD, sporadic, familial, kuru, NvCJD, BSE, contaminate meat products, epidemiology, cannabilism, cornal transplants, iatrogenic disease epidemiology, prion diseases, prion genetics and biochemistry, zoonotic disease.

127. Polak, Miroslaw P.; Rozek, Wojciech; Zmudzinski, Jan F. Monitoring BSE. [BSE monitoring.] Medycyna Weterynaryjna. 2002; 58 (4): 265-266. In Polish.
     NAL call no. 41.8 M463
     Descriptors: review paper, active surveillance principles, bovine spongiform encephalopathy, Switzerland, EU, Poland, diagnostic tests.

128. Polak, M.P.; Rozek, W.; Zmudzinski, J.F. Monitoring BSE w Polsce. [BSE monitoring in Poland.] Medycyna Weterynaryjna. 2002; 58 (5): 344-347. In Polish.
     NAL call no. 41.8 M463
     Descriptors: diagnostic tests, Prionics-Check(R), monitoring of BSE, EU, cattle, randon sampling.

129. Poser, Charles M. Notes on the history of the prion diseases. Part I. Clinical Neurology and Neurosurgery. 2002 Jan; 104(1): 1-9 ISSN: 0303-8467.
     Abstract: The astute observation by William Hadlow, an American veterinary neuropathologist of the similarity between the histopathology of kuru, an obscure disease of the primitive tribe in New Guinea, and scrapie of sheep, was the first clue to the etiology of the transmissible spongiform encephalopathies (TSE). The knowledge that scrapie was transmissible but only after an unusually long incubation period, that the causative agent was highly resistant to heat and formalin, and that it seemed to be able to replicate in the absence of nucleic acid, eventually led to the discovery of the prion by Stanley Pruisner and the still controversial protein-only hypothesis of etiology of the TSE.
     Descriptors: kuru, scrapie, transmissible spongiform encephalopathies, prion disease theory.

130. Poser, Charles M. Notes on the history of the prion diseases. Part II. Clinical Neurology and Neurosurgery. 2002 May; 104(2): 77-86 ISSN: 0303-8467
     Abstract: The protein-only theory of transmission of the prion diseases remains controversial. Other mechanisms such as the virus, virino, and viroid hypotheses are still under consideration. All these fit in the concept of 'slow' infections that had been proposed in 1954 by Bjorn Sigurdsson, an Icelandic pathologist. Regardless of the exact mode of infection, the presence of prions in the brain has served to unite Creutzfeldt-Jakob disease (CJD), the Gerstmann-Straussler-Scheinker syndrome and fatal familial insomnia, as well as scrapie and a number of other animal diseases, into a single pathological entity, the transmissible spongiform encephalopathies. The appearance of bovine spongiform encephalopathy in the United Kingdom and its putative relationship to new variant CJD, have put a new and unpredictable light on these unusual and uncommon diseases.
     Descriptors: disease transmission theories, BSE, Creutzfeldt-Jakob disease, CJD, the Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, scrapie, NvCJD, pathological agent.

131. Poser, S.. BSE - Eine Ursache fur die Creutzfeldt-Jakob-Krankheit? [BSE - A cause for Creutzfeldt-Jakob disease?] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 311 ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: BSE, bovine spongiform encephalopathy, transmission, diagnosis, epidemiology, biological markers, cattle, iatrogenic disease, risk factors, France, Germany, Great Britain, Ireland.

132. Poser, S.; Zerr, I.; Felgenhauer, K. Die neue Variante der Creutzfeldt-Jakob-Krankheit. [New variant Creutzfeldt-Jakob disease]. Deutsche Medizinische Wochenschrift. 2002 Feb 15; 127(7): 331-4 ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: NvCreutzfeldt-Jakob Disease, epidemiology, BSE, bovine spongiform encephalopathy, diagnosis, transmission, contaminated meat, human risk factors, incidence of disease.

133. Ramsay, A. R145: Evolution of a TSE diagnostic antibody. Research in Veterinary Science. April, 2002; 72 (Supplement A): 48. 56th Annual Conference of the Association of Veterinary Teachers and Research Workers on Current Topics in Veterinary Science, Scarborough, England, UK, March 25-27, 2002.
     NAL call no. 41.8 R312
     Descriptors: transmissible spongiform encephalopathies, diagnostic test, R145 antibody, development.

134. Redman, C.A.; Coen, P.G.; Matthews, L.; Lewis, R. M.; Dingwall, W.S.; Foster, J.D.; Chase-Topping, M. E.; Hunter, N.; Woolhouse, M. E. J. Comparative epidemiology of scrapie outbreaks in individual sheep flocks. Epidemiology and Infection 2002 Jun; 128(3): 513-21.
     Abstract: Data recording the course of scrapie outbreaks in 4 sheep flocks (2 in Cheviot sheep and 2 in Suffolks) are compared. For each outbreak the data on scrapie incidence and sheep demography and pedigrees cover periods of years or decades. A key finding is that the incidence of clinical cases peaks in sheep 2-3 years old, despite very different forces-of-infection. This is consistent with age-specific susceptibility of sheep to scrapie, as has been reported for cattle to bovine spongiform encephalopathy and for humans to variant Creutzfeldt-Jakob disease. Scrapie incidence was higher in ewes than rams and at certain times of years, though these effects were not consistent between flocks. There was no evidence for high levels of vertical transmission.
     NAL call no. RA651.A1E74
     Descriptors: scrapie, sheep, disease outbreaks, vertical transmission, male and female comparisons.

135. Resende, C.; Parham, S.N.; Tinsley, C.; Ferreira, P.; Duarte, J.A.B.; Tuite, M.F. The Candida albicans Sup35p protein (CaSup35p): function, prion-like behaviour and an associated polyglutamine length polymorphism. Microbiology. 2002, 148: 4. ISSN: 1049-1060.
     NAL call no. QR1 J64
     Descriptors: prions, yeast protein, comparison, biochemistry, Sup35pprotein.

136. Riley, Maria Louise; Leucht, Christoph; Gauczynski, Sabine; Hundt, Christoph; Brecelj, Martina; Dodson, Guy; Weiss, Stefan High-level expression and characterization of a glycosylated covalently linked dimer of the prion protein. Protein Engineering. June, 2002; 15 (6): 529-537.
     NAL call no. TP248 P77P763
     Descriptors: prion protein dimers, scrapie prion protein PrPSc, PrPc, human dimeric form, digestibility by proteinase, life cycle of proteins.

137. Rosted, Palle; Jorgensen, Viggo Kragh. Prion strain causing bovine spongiform encephalopathy (BSE) in cattle. Annals of Surgery. 2002 Feb; 235(2): 311. ISSN: 0003-4932.
     Descriptors: cats diseases, prion diseases, BSE, cattle.

138. Sabate, Raimon; Estelrich, Joan. Aggregation characteristics of ovalbumin in beta-sheet conformation determined by spectroscopy. Biopolymers. 2002; 67(2): 113-20 ISSN: 0006-3525.
     Abstract: Protein misfolding and aggregation are involved in a number of the so-called "conformational" diseases (e.g., transmissible spongiform encephalopathies and Alzheimer disease). The development of rational strategies to interfere with aggregation is a potential therapeutic approach that requires complete knowledge of the aggregation process. We studied the aggregation of ovalbumin in beta-sheet conformation using mainly the spectral changes in the spectra of two dyes (Congo Red and pinacyanol) caused by the aggregates. We assumed a linear model of polymerization that fit to the experimental data. The critical aggregation constant, concentration of half-aggregation, nucleation parameter, growth parameter, and number of aggregation and free energy changes (total and per residue) were determined as aggregation-related parameters. Beta-Ovalbumin aggregates in a cooperative way. Moreover, the differences between such parameters obtained with Congo Red and pinacyanol suggest that each dye interacts with the protein in its own way.
     NAL call no. 381 B524
     Descriptors: protein structure, misfolding, prions, transmissible spongiform encephalopathies.

139. Sasaki, Kensuke; Dohura, Katsumi; Ironside, James W; Iwaki, Toru. Increased clusterin (apolipoprotein J) expression in human and mouse brains infected with transmissible spongiform encephalopathies. Acta Neuropathologica (Berl). 2002 Mar; 103(3): 199-208. ISSN: 0001-6322.
     Abstract: Clusterin (apolipoprotein J), a multifunctional protein involved in amyloidogenesis in Alzheimer's disease, was studied immunohistochemically in both human transmissible spongiform encephalopathies (TSEs) and a mouse model of human TSE. Clusterin immunoreactivity was co-localized with plaque-type deposits but not with punctate-type prion protein (PrP) deposits in human TSEs. On the other hand, clusterin-positive astrocytes were readily demonstrated in the regions of punctate PrP deposits, but not around plaque PrP deposits despite the presence of surrounding astrocytes. Clusterin expression in astrocytes was not disease specific, but the punctate immunoreactivity for clusterin was more prominently demonstrated in TSEs with punctate PrP deposits. Serial analysis in the mouse model of human TSE revealed that clusterin expression in astrocytes was enhanced in the lesions with punctate-type PrP deposits during the disease progression. Thus, the induction of clusterin expression in astrocytes could be more enhanced by punctate-type PrP deposits than by plaque-type deposits. The clusterin molecules co-localized in plaque PrP deposits might be derived not from surrounding astrocytes but from other resources such as cerebrospinal fluid and blood plasma, both of which contain clusterin in significant amounts. Taken together with previously reported findings of the anti-amyloidogenic property in clusterin, our findings suggest that clusterin may be induced as one of the important molecules participating in the neurodegeneration caused by abnormally deposited PrP.
     Descriptors: protein analysis, immunohistochemical assay, TSE's mouse model, plaque PrP deposites, neurodegeneration process.

140. Scholz, Roland. 25 Thesen gegen die Behauptung, BSE und vCJK seien oral ubertragbare Infektionskrankheiten und BSE gefahrde die menschliche Gesundheit. [25 theses against the assertion that BSE and vCJD are orally transmissible infectious diseases and endanger human health]. Deutsche Medizinische Wochenschrift. 2002 Feb 15; 127(7): 341-3. ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: Nv Creutzfeldt-Jakob Disease, bovine spongiform spongiform encephalopathy, transmission theories, cattle, epidemiology, meat products, human health food risks, risk factors.

141. Schreuder, B.E.C.; Wever, C.J.G. Waar komt BSE in Nederland vandaan? [The possible origin of BSE in the Netherlands.] Tijdschrift voor Diergeneeskunde. 2002, 127: 2, 40-50; 29 ref. ISSN: 0040-7453. In Dutch with an English summary.
     Abstract: It is only in the last 5 years that the Netherlands has been confronted with cases of bovine spongiform encephalopathy (BSE). The cases diagnosed to date have not been clearly linked to imports from the United Kingdom. This article describes the various possible explanations for the Dutch cases. The risk factors involved, have either a connection with imported BSE, local origin of BSE, or both. These factors can also be divided into introductory risk and propagation risk, terms that were also used in an EU risk assessment study. Research at ID-Lelystad since the early 1990s and at IKC-Ede has tried to assess the relative importance of the various risk factors, the results of which are discussed in this paper. The paper does not deal with the specifics of the cases diagnosed to date, because of the absence of an in-depth epidemiological investigation, but provides a general assessment of the risk factors that might have played a role. Important factors have been, in addition to the initial imports of cattle and meat and bone-meal from the UK, the continuing imports from other countries with covert BSE and the cross-contamination within the animal feed production lines. Emphasis is on the period of the early and mid-1990s, the period in which most calves with diagnosed BSE were born.
     NAL call no. 41.8 T431
     Descriptors: bovine spongiform encephalopathy, BSE, history of disease introduction, animal feeds, Netherlands, importance of risk factors leading to disease introduction, importation of infected animals, animal feed formulations.

142. Schulz-Schaeffer, Walter J. BSE und variante CJK. Von den Schwierigkeiten, ein neues Krankheitsprinzip zu etablieren. [BSE and variant CJD: about the difficulties to establish a new pathogenetic principle.] Deutsche Medizinische Wochenschrift 2002 Feb 15; 127(7): 344-6. ISSN: 0012-0472. In German.
     NAL call no. 448.8 D48
     Descriptors: bovine spongiform encephalopathy, cattle, consumer food safety, Europe, epidemiology, NvCreutzfeldt-Jakob Disease, cattle, hamsters, mice, primates, prions, risk factors, scrapie, time factors.

143. Schutt-Abraham, Ingrid. BSE-Praventivmassnahmen bei der Schlachtung von Rindern. [Measures preventing BSE-contamination during the slaughter of cattle.] Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 125-30. ISSN: 0005-9366. In German.
     Abstract: SRM-regulations and the prohibition of pithing have removed major risks of spreading BSE-infection. Traditional slaughter technology, especially captive bolt stunning, head handling and carcass splitting nevertheless still provide non-negligible risks for contamination with the BSE-agent if present in cattle, and should therefore be replaced by safer techniques. However, alternative methods like electrical stunning or removal of the spinal cord prior to splitting the carcass cannot yet be considered a reliable and practical option. Surface contamination could be prevented altogether by abandoning the practice of carcass splitting and by removing the vertebral column while still connected to the head, although this would result in disadvantages for post mortem inspection.
     NAL call no. 41.8 B45
     Descriptors: cattle, slaughter, captive bolt, head handling, carsass splitting, contamination with CNS tissue, alternative methods, spinal cord removal.

144. Sethi, Shneh; Lipford, Grayson; Wagner, Hermann; Kretzschmar, Hans. Postexposure prophylaxis against prion disease with a stimulator of innate immunity. Lancet. 2002 Jul 20; 360(9328): 229-30 ISSN: 0140-6736.
     Abstract: The absence of an immune response to prions--the infectious agents of scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease--might be related to the fact that these agents do not contain nucleic acids. We aimed to use CpG oligodeoxynucleotides, which have been shown to stimulate innate immunity, as a form of postexposure prophylaxis in mice. We inoculated healthy mice with brain homogenates from mice infected with the RML scrapie prion, and then injected CpG oligodeoxynucleotides. This postexposure prophylaxis with CpG oligodeoxynucleotides resulted in 38% longer survival times than treatment with saline (p<0.0001), or even longer after repeated application. The explanation for this finding remains to be elucidated, but the most likely is stimulation of TLR9-expressing cells of the innate immune system such as macrophages, monocytes, and dendritic cells. CpG oligodeoxynucleotides have not been shown to have adverse effects to human health and could therefore be considered as a therapeutic choice in postexposure prophylaxis.
     NAL call no. 448.8 L22
     Descriptors: infectious agents, scrapie, bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease, mouse model, CpG oligodeoxynucleotides, stimulation of immunity.

145. Shaked, Yuval; Engelstein, Roni; Gabizon, Ruth. The binding of prion proteins to serum components is affected by detergent extraction conditions. : Journal of Neurochemistry. 2002 Jul; 82(1): 1-5 ISSN: 0022-3042.
     Abstract: As many GPI anchored proteins, PrP(C) and its abnormal conformer PrP(Sc), are inserted into membrane microdomains known as rafts. Upon raft disruption, PrP(C) becomes soluble, while PrP(Sc) aggregates into insoluble structures. It was recently published that, as opposed to PrP(C), PrP(Sc), as well as its protease resistant core PrP27-30, can bind specifically to plasminogen and other serum components. These findings were suggested to have important physiological implications in transmissible spongiform encephalopathies (TSE) diagnosis and pathogenesis. In this work, we show that the binding of PrP(Sc) or PrP 27-30 to serum proteins occurs only at specific detergent combinations, in which disease associated PrPs are present in aggregated structures. At detergent conditions in which rafts are intact, it is actually PrP(C.) that binds to blood proteins, albeit not directly, but through neighboring rafts components. Our results therefore indicate that the binding of PrP(Sc) to blood components has no physiological relevance.
     NAL call no. QP351 J6
     Descriptors: raft disruption, PrPSc, factors affecting transmissibility, diagnosis, and pathogenesis, serum protein binding, affects of detergent combinations, physiological relevance.

146. Simak, Jan; Holada, Karel; D'Agnillo, Felice; Janota, Jan; Vostal, Jaroslav G. Cellular prion protein is expressed on endothelial cells and is released during apoptosis on membrane microparticles found in human plasma. Transfusion. 2002 Mar; 42(3): 334-42 ISSN: 0041-1132.
     Abstract: BACKGROUND: Blood and plasma of animals experimentally infected with transmissible spongiform encephalopathies (TSEs) can transmit TSE infection by transfusion. A conformational isoform of prion protein (PrPsc) is believed to be the TSE-infectious agent that propagates by converting the cellular prion protein (PrPc) to additional molecules of PrPsc. In orally infected animals, PrPsc accumulates in intestinal endothelial cells. In blood, two thirds of PrPc resides in plasma, but its source is not known. STUDY DESIGN AND METHODS: The expression of PrPc in cultured human umbilical vein endothelial cells (HUVECs) was studied using flow cytometry, immunoblotting, and RT-PCR. Flow cytometry was used to characterize endothelial membrane microparticles (MPs) in cell culture supernatants and in normal human plasma. RESULTS: HUVECs and bovine aorta endothelial cells express PrPc. The number of surface PrPc molecules per cell in HUVECs was 58,000 +/- 2,800. The induction of apoptosis in HUVECs led to a marked release of membrane MPs (60,000-80,000 MPs/10(3) cells) that expressed PrPc and other endothelial antigens. The presence of endothelial cell-derived MPs expressing PrPc was demonstrated in platelet-free human plasma. CONCLUSION: Endothelial cell apoptosis is associated with the release of PrPc-positive MPs. These MPs contribute to the PrPc pool in plasma and may have a role in disseminating TSE infectivity in blood.
     Descriptors: experimental infections, TSEs, transmissible spongiform encephalopathies, PrPsc, cultured human umbilical vein endothelial cells, membrance microparticles carriers.

147. Singh, Neena; Gu, Yaping; Bose, Sharmila; Kalepu, Sudheera; Mishra, Ravi Shankar; Verghese, Susamma. Prion peptide 106-126 as a model for prion replication and neurotoxicity. Frontiers of Bioscience Computer File: a Journal and Virtual Library. 2002 Apr 1; 7: a60-71. ISSN: 1093-4715.
     Abstract: Prion diseases or transmissible spongiform encephalopathies are neurodegenerative disorders that are genetic, sporadic, or infectious. The pathogenetic event common to all prion disorders is a change in conformation of the cellular prion protein (PrPC) to the scrapie isoform (PrPSc), which, unlike PrPC, aggregates easily and is partially resistant to protease digestion. Although PrPSc is believed to be essential for the pathogenesis and transmission of prion disorders, the mechanism by which PrPSc deposits cause neurodegeneration is unclear. It has been proposed that in some cases of prion disorders, a transmembrane form of PrP, termed CtmPrP may be the mediator of neurodegenerative changes rather than PrPSc per se. In order to understand the underlying cellular processes by which PrPSc mediates neurodegeneration, we have investigated the mechanism of neurotoxicity by a beta-sheet rich peptide of PrP in a cell model. We show that exposure of human neuronal cell lines NT-2 and M17 to the prion peptide 106-126 (PrP106-126) catalyzes the aggregation of endogenous cellular prion protein (PrPC) to an amyloidogenic form that shares several characteristics with PrPSc. Intracellular accumulation of these PrPSc-like forms upregulates the synthesis of CtmPrP, which is proteolytically cleaved in the endoplasmic reticulum and the truncated C-terminal fragment is transported to the cell surface. In addition, we have isolated mutant NT-2 and neuroblastoma cells that are resistant to toxicity by PrP106-126 to facilitate further characterization of the biochemical pathways of PrP106-126 neurotoxicity. The PrP106-126-resistant phenotype of these cells could result from aberrant binding or internalization of the peptide, or due to an abnormality in the downstream pathway(s) of neuronal toxicity. Thus, our data suggest that PrPSc aggregation occurs by a process of 'nucleation' on a pre-existing 'seed' of PrP. Furthermore, the PrP106-126-resistant cells reported here will provide a unique opportunity for identifying the cellular and biochemical pathways that mediate neurotoxicity by PrPSc.
     Descriptors: Prion diseases, transmissible spongiform encephalopathies, PrPSc, human neuronal call lines Nt-2, M17, PrP 106-126, process is nucleation on a pre-existing seed.

148. Somerville, R.A.; Oberthur, R.C.; Havekost, U.; Macdonald, F.; Taylor, D.M.; Dickinson, A.G. Characterization of thermodynamic diversity between transmissible spongiform encephalopathy agent strains and its theoretical implications. J Biol Chem. Mar 29, 2002. v. 277 (13) p. 11084-11089. ISSN: 0021-9258
     DOI: 10.1074/jbc.M111766200
     Abstract: Some transmissible spongiform encephalopathy (TSE) (or "prion") strains, notably those derived from bovine spongiform encephalopathy, are highly resistant to total inactivation by heat. When three TSE strains derived from sheep with scrapie were heated, little inactivation took place at low temperatures, but at higher temperatures, considerable inactivation occurred. The temperature at which substantial inactivation first occurred varied according to TSE strain, and it was calculated to be 70 degrees C for the 22C strain, 84 degrees C for ME7, and 97 degrees C for 22A by fitting the data to a model based on competition between a destructive and a protective reaction. However, PrP(Sc) from mice infected with a range of TSE strains retained similar resistance to proteinase K digestion after heating to below or above these temperatures, showing that the properties of PrP(Sc) responsible for proteinase resistance do not correlate with those conferring thermostability on the TSE agent. The simplest explanation of these data is that the causal agent contains a macromolecular component that is structurally independent of the host, that it varies covalently between TSE strains, and that it is protected by other macromolecular components. The model is in accord with the virino hypothesis, which proposes a host-independent informational molecule protected by the host protein PrP.
     NAL call no. 381 J824
     Descriptors: TSE, scrapie agents, strains, isolation, sheep, strain differences, heat inactivation, temperature effects, infectivity, mouse model, glycoproteins, proteolysis, animal prion proteins.

149. Soto, Claudio; Saborio, Gabriela P.; Anderes, Laurence. Cyclic amplification of protein misfolding: Application to prion-related disorders and beyond. Trends in Neurosciences. August, 2002; 25 (8): 390-394.
     DOI: 10.1016/S0166-2236(02)02195-1
     NAL call no. RC321 T74
     Descriptors: methods, amplify cyclically misfolded proteins in vitro, PMCA, potential diagnostic technique, transmissible spongiform encephalopathies, new technology, applications.

150. Spraker, T.R.; Zink, R.R.; Cummings, B.A.; Wild, M.A.; Miller, M.W.; O'Rourke, K.I. Comparison of histological lesions and immunohistochemical staining of proteinase-resistant prion protein in a naturally occurring spongiform encephalopathy of free-ranging mule deer (Odocoileus hemionus) with those of chronic wasting disease of captive mule deer. Veterinary Pathology. 2002 January; 39(1): 110-119 ISSN: 0300-9858.
     NAL call no. 41.8 P27
     Descriptors: mule deer, comparison between captive and free ranging, PrPRES, chronic wasting disease, TSE, histological lesions and immunohistochemical staining.

151. Spraker, T.R.; O' Rourke, K. I.; Balachandran, A.; Zink, R.R.; Cummings, B.A.; Miller, M.W.; Powers, B.E. Validation of monoclonal antibody F99/97.6.1 for immunohistochemical staining of brain and tonsil in mule deer (Odocoileus hemionus) with chronic wasting disease. Journal of Veterinary Diagnostic Investigation. 2002, 14: 1, 3-7; 18 ref.
     NAL call no. SF774.J68
     Descriptors: deer, transmissible spongiform encephalopathies, brain and tonsil tissue staining, diagnosis, chronic wasting disease, immunohistochemical staining method, wildlife,Colorado, Montana.

152. Staufenbiel, Rudolf; Hamalainen, Mira. Zur klinischen Diagnostik der BSE. [The clinical diagnosis of BSE.] Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 99-105 ISSN: 0005-9366. In German.
     Abstract: Diagnosis of Bovine spongiform encephalopathy (BSE) is confirmed by specified laboratory methods on brain material. On the other hand clinical signs of manifest BSE are quite obvious. The first part of this paper describes case histories, clinical signs, laboratory findings and the most common differential diagnoses. On the basis of the data of actual prevalence in Germany, the role of clinical examination in eradication of BSE is dealt in the second part. Clinical diagnosis is a very sensitive and specific method when there is a high prevalence. According to the data from December 2000 to November 2001 prevalence in Germany was beyond 1 BSE case per 100,000 cattle or 3 cases per 100,000 cows. This very low prevalence decreases rapidly sensitivity and specificity of the diagnosis made by clinical examination. Therefore the main focus of field-diagnostics has to be laid on specified laboratory diagnostic methods. On the other hand prevalence of BSE-positive cattle is distinctly higher in the group of animals slaughtered in cases of illness or emergency than in cattle slaughtered on the regulatory bases. Nevertheless every veterinary practitioner should be aware of the clinical picture of BSE, clinical examination-routine and differential diagnosis, because occurrence of BSE is still possible in any dairy herd. At the moment it is not possible to make any statement if eradication of BSE can be reached in future.
     NAL call no. 41.8 B45
     Descriptors: cattle, bovine spongiform encephalopathy, case histories, clinical signs, lab findings, differential diagnoses, control and eradication.

153. Stewart, Gordon T.More on BSE/vCJD. Journal of the Royal Society of Medicine 2002 Feb; 95(2): 112. ISSN: 0141-0768.
     NAL call no. 448.9 R814
     Descriptors: NvCreutzfeldt Jakob syndrome, epidemiology, bovine spongiform encephalopathy, cattle, humans transmission.

154. Sugaya, Makoto; Nakamura, Koichiro; Watanabe, Takahiro; Asahina, Akihiko; Yasaka, Nami; Koyama, Yoh Ichi; Kusubata, Masashi; Ushiki, Yuko; Kimura, Kumiko; Morooka, Akira; Irie, Shinkichi; Yokoyama, Takashi; Inoue, Keiichi; Itohara, Shigeyosi; Tamaki, Kunihiko. Expression of cellular prion-related protein by murine Langerhans cells and keratinocytes. Journal of Dermatological Science. February, 2002; 28 (2): 126-134. ISSN: 0923-1811.
     DOI: 10.1016/S0923-1811(01)00160-8
     Abstract: Transmissible spongiform encephalopathies are characterized by the accumulation of a proteinase-resistant isoform of the cellular prion-related protein (PrPc) within the central nervous system (CNS). The accumulation of scrapie-associated PrP (PrPSc) within cells of the lymphoreticular system prior to its accumulation in the CNS is regarded as important for the development of neurological diseases after peripheral inoculation. Little, however, is known as to which cells are the targets for peripheral inoculation. Here, the presence of PrPc on murine Langerhans cells (LC), dendritic cells in the skin and mucosa, and keratinocytes (KC) is demonstrated by immunohistochemical staining, Western-blotting and FACS analysis. The expression of PrPc mRNA in freshly purified LC and KC was also detected by reverse transcriptase-polymerase chain reaction. The expression of PrPc on LC was slightly increased during culture. These data suggest that LC and KC may be the targets for peripheral infection with prions.
     Descriptors: Keratinocytes metabolism, Langerhans cells metabolism, phosphoprotein phosphatase metabolism, base sequence, gene expression, immunohistochemistry, mouse model inbred-BALB-C, inbred-C57B, knockout mice, phosphoprotein-phosphatase genetics, prion disease genetics, prion diseases metabolism, transmissible spongiform encephalopathies.

155. Supattapone, Surachai; Nishina, Koren; Rees, Judy R. Pharmacological approaches to prion research. Biochemical Pharmacology. 2002 Apr 15; 63(8): 1383-8 ISSN: 0006-2952
     Abstract: The "protein-only" mechanism by which infectious agents of prion diseases such as Creutzfeldt-Jakob disease and bovine spongiform encephalopathy replicate remains undetermined. The identification of several distinct classes of prion inhibitors has created an opportunity to investigate the mechanism of prion formation using pharmacological tools. These new inhibitors include substituted tricyclic derivatives, tetrapyrrole compounds, cysteine protease inhibitors, branched polyamines, and specific antibodies. Each inhibitor class contains at least one active compound that inhibits prion propagation in cell culture at sub-micromolar concentrations and several structurally related, inactive compounds. Work with branched polyamines and specific antibodies has already provided insight into the kinetics and cell biology of endogenous prion clearance mechanisms. Other anti-prion compounds do not appear to bind directly to the prion protein. Detailed investigation of the mechanism of drug action of these compounds may lead to the identification of novel prion propagation factors.
     NAL call no. 396.8 B52
     Descriptors: prion diseases, infectious agents, CJD, BSE, bovine spongiform encephalopathy, tricyclic derivatives, tetrapyrroles, cysteine protease inhibitors, branched polyamines, specific antibodies, drug actions.

156. Sy, Man Sun; Gambetti, Pierluigi; Wong, Boon Seng. Human prion diseases. Medical Clinics of North America. May, 2002; 86 (3): 551-571.
     NAL call no. RC660 S95 F&N B-4343
     Descriptors: BSE, Creutzfeldt Jakob Disease, NvCreutzfeldt Jakob Disease, kuru, other transmissible or genetic spongiform encephalopathies.

157. Takekida, Kaori; Kikuchi,Yutaka; Yamazaki, Takeshi; Horiuchi, Motohiro; Kakeya, Tomoshi; Shinagawa, Morikazu; Takatori, Kosuke; Tanimura, Akio; Tanamoto, Ken-Ichi; Sawada, Jun ichi. Quantitative analysis of prion protein by immunoblotting. Journal of Health Science. June, 2002; 48 (3): 288-291.
     URL: http://jhs.pharm.or.jp/data/48(3)/48_288.htm
     Descriptors: TSE, transmissible spongiform encephalopathy, PrPSc, quantitiative analysis technique, immunoblotting, densitometry data, bovine PrPC, deglycosylated forms.

158. Taylor, David. Inactivation of the BSE agent. Comptes Rendus Biologies. Janvier, 2002; 325 (1): 75-76.
     DOI: 10.1016/S1631-0691(02)01386-0
     NAL call no. Q2 C6
     Descriptors: BSE, resistant to inactivation procedures, sodium hypochlorite solutions and autoclaving combined, mouse passaged agent, acid treated bone.

159. Tibayrenc, Michel; Mas, Coma Santiago; Piffaretti, Jean Claude; Struelens, Marc. The European Centre for Infectious Diseases: An adequate response to the challenges of bioterrorism and major natural infectious threats. Infection Genetics and Evolution. May, 2002; 1 (3): 179-181.
     DOI: 10.1016/S1567-1348(02)00035-7
     Descriptors: government center, Europe, organization to address invasive diseases such as bovine spongiform encephalopathy, other zoonotic diseases.

160. Tompa, Peter; Tusnady, Gabor E; Friedrich, Peter; Simon, Istvan The role of dimerization in prion replication. Biophysical Journal. April, 2002; 82 (4): 1711-1718.
     URL: http://www.biophysj.org/cgi/content/abstract/82/4/1711
     Descriptors: prion disease, conformational transition, dimerization role in prion replication, intramolecular disulfide bridge, theory to explain prion conformational changes.

161. Tuzi, Nadia L.; Gall, Elaine; Melton, David; Manson, Jean C. Expression of doppel in the CNS of mice does not modulate transmissible spongiform encephalopathy disease. Journal of General Virology. 2002 Mar; 83(Pt 3): 705-11. ISSN: 0022-1317.
     Abstract: Late onset ataxia reported in three independently derived PrP null lines of mice has been attributed to the overexpression of the doppel protein in the CNS of these mice rather than to the loss of PrP. The central role of PrP in the transmissible spongiform encephalopathies (TSEs), the proximity of the gene which encodes doppel (Prnd) to the PrP gene (Prnp) and the structural similarity shared by PrP and doppel have led to the proposition that ataxia which develops during TSE disease could, in part, be due to doppel. In order to address this hypothesis, we have crossed our two inbred lines of PrP null mice, which either express (RCM) or do not express (NPU) the Prnd gene in the CNS, with mice expressing two Prnp(a[108F189V]) alleles of the PrP gene. We have found that the TSE infection does not influence the level of expression of Prnd in the CNS at the terminal stages of disease. Moreover, we have demonstrated that the level of expression of Prnd in the CNS has no influence on the incubation period, vacuolar pathology nor amount or distribution of deposition in the brains of the TSE-infected mice. Doppel has therefore no apparent influence on the outcome of TSE disease in transgenic mice, suggesting it is unlikely to be involved in the naturally occurring TSE diseases in other species.
     NAL call no. QR360.A1J6
     Descriptors: PrP, doppel protein, mouse models, TSE.

162. VandeVondele, Joost; Colombo, Maria Carola; Laio, Alessandro; Guidoni, Leonardo; Rothlisberger, Ursula. QM/MM study of the copper binding site of prion protein. Biophysical Journal. January, 2002; 82 (1 Part 2): 487a. 46th Annual Meeting of the Biophysical Society, San Francisco, California, USA, February 23-27, 2002
     NAL call no. 442.8 B5238
     Descriptors: prion proteins, molecular binding structure, copper, QM/MM study.

163. Van't Hooft, A. J.G. BSE en de soortspecifieke barriere. [BSE and species specificity.] Tijdschrift voor Diergeneeskunde. 2002 Mar 1; 127(5): 175 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: Creutzfeldt Jakob syndrome, etiology, bovine spongiform encephalopathy, transmission, species specificity.

164. Wahlstrom, Helene; Elvander, Marianne; Engvall, Anders; Vagsholm, Ivar Risk of introduction of BSE into Sweden by import of cattle from the United Kingdom. Preventive Veterinary Medicine. 25 June, 2002; 54 (2): 131-139.
     DOI: 10.1016/S0167-5877(02)00019-3
     NAL call no. SF601.P7
     Descriptors: cattle, BSE risks, transported cattle from UK to Sweden, 1980, surveillance system.

165. Wiemer, Udo. Veterinarrechtliche Schutzmassnahmen im Hinblick auf BSE. [Protective measures taken with regard to BSE under veterinary law]. Berliner und Munchener Tierarztliche Wochenschrift. 2002 Mar-Apr; 115(3-4): 134-9 ISSN: 0005-9366. In German.
     Abstract: BSE was established for the first time in 1986 as a separate disease complex. Since 1989 measures to protect human and animal health have been adopted at Community level and under German law. The article describes the most important provisions governing the prevention, control and eradication of TSE. It addresses in detail the ban on feeding, active monitoring of BSE, active monitoring of small ruminants, measures taken after the detection of BSE, the removal and destruction of specified risk material and briefly addresses trade bans and restrictions.
     NAL call no. 41.8 B45
     Descriptors: BSE, transmissible spongiform encephalopathies, prevention, control, eradication, animal feeds, small ruminants, trade base and restructions.

166. Will, R.G. Variant Creutzfeldt-Jakob disease - How new is new?. Journal of Neurology Neurosurgery and Psychiatry. March, 2002; 72 (3): 285-286.
     URL: http://jnnp.bmj.com/cgi/content/extract/72/3/285
     Descriptors: BSE, bovine spongiform encechalopathy, NvCJD, pathogenesis.

167. Wrathall, A.E.; Brown, K.F.D.; Sayers, A.R.; Wells, G.A.H.; Simmons, M.M.; Farrelly, S.S.J.; Bellerby, P.; Squirrell, J.; Spencer, Y.I.; Wells, M.; Stack, M.J. Studies of embryo transfer from cattle clinically affected by bovine spongiform encephalopathy (BSE). Veterinary Record. Mar 23, 2002. v. 150 (12) p. 365-378. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: cattle embryo transfer, bovine spongiform encephalopathy, disease transmission, washing, semen, fertilizing ability, gametogenesis, prion proteins, genotypes, infectivity, UK.

168. Wuthrich, Kurt; Calzolai, Luigi; Guntert, Peter; Luhrs, Thorsten; Lysek, Dominik; Schorn, Christian; Von Schroetter, Christine; Zahn, Ralph. Species variation of the three-dimensional prion protein structure in the cellular form. Biophysical Journal. January, 2002; 82 (1 Part 2): 169a. 46th Annual Meeting of the Biophysical Society, San Francisco, California, USA, February 23-27, 2002
     NAL call no. 442.8 B5238
     Descriptors: prion proteins, protein structions and conformation, species variation.

169. Zaaijer, H. L. Interpretatie van de toename van boviene spongiforme encefalopathie buiten Groot-Brittannie. [Interpretation of the increase in bovine spongiform encephalopathy outside Great Britain.] Nederlands Tijdschrift voor Geneeskunde 2002 Apr 20; 146(16): 748-50. ISSN: 0028-2162. In Dutch.
     Abstract: Outside Great Britain, the number of clinical cases of bovine spongiform encephalopathy (BSE) seems to be rising. It is unclear whether this increase is real, or whether it is caused by improved recognition and improved registration of BSE. A strict and independent control of the implementation of measures intended to keep human food free of the BSE agent is imperative.
     Descriptors: bovine spongiform encephalopathy, cattle, consumer food safety, Europe, epidemiology, Great Britian, control measures.

170. Zentek, J.; Oberthur, R.C.; Kamphues, J.; Kreienbrock, L.; Flachowsky, G.; Coenen, M.. Futtermittel tierischer Herkunft als mogliche Verbreitungsursache fur die bovine spongiforme Enzephalopathie (BSE) in Deutschland. 2. Mitteilung: Einschatzung des Verbreitungsrisikos uber Mischfutter. [Animal-derived feedstuffs as possible vectors for bovine encephalopathy (BSE) in Germany. Part 2: Assessment of vector risk for compounded feed.] . DTW. Deutsche Tierarztliche Wochenschrift. 2002 Feb: 109 (2): 43-51 ISSN: 0341-6593. In German.
     Abstract: Specific conditions and practices of cattle feeding in Germany have to be taken into account for assessing the risk of feed born transmission of bovine spongiform encephalopathy, especially regarding the situation before the year 2000 when specific directives were introduced for feed production. The present retrospective epidemiological study includes data on feed production and the estimated amount of animal derived feedstuffs for the production of compounded feed for cattle. Risk assessment was performed based on the 'reproduction rate' (R0), that is defined as the estimated number of infections resulting from the processing of brain and spinal cord of BSE affected cattle that is recycled to bovines via feed. Under the conditions as given in Germany until the year 2000 the reproduction rate of BSE via the inclusion of animal derived feedstuffs in compounded feed production for cattle was estimated to be 1.1. Thus, it can be expected that BSE could be reproduced in the system, but with comparatively low efficiency. The expected incidence of BSE should be considerably lower compared to the situation during the 90th in the UK, due to the markedly lower recycling rate of animal protein in cattle feeding. Animal fat could have been a significant factor for BSE transmission due to contamination by proteinaceous brain and spinal cord material during the production process. The relative significance of fat containing feedstuffs for BSE transmission could have been higher in Germany compared to the situation in the UK where meat and bone meal was produced under different conditions and frequently used in higher proportions as an ingredient for compounded feed for ruminants.
     NAL call no. 41.8 D482
     Descriptors: cattle, feeding and feeds, BSE, disease transmission risks, animal derived feed additives, Germany.

171. United States. General Accounting Office. Mad cow disease: improvements in the animal feed ban and other regulatory areas would strengthen U.S. prevention efforts. Report to Congressional Requestors, Washington, D.C.: GAO, January 2002. 59 p.
     URL: www.gao.gov/new.items/d02183.pdf
     Descriptors: Bovine spongiform encephalopathy, animal feed, prevention strategies, public and animal health risks, animal feed ban compliance, probablistic simulation model, detection in imported animal products, economic impacts of the disease, recommendations to USDA and FDA, GAO report.

2001

1. Abbott, A. BSE fallout sends shock waves through Germany. Nature. 2001 Jan 18. 409(6818): 275 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: Bovine spongiform encephalopathy, meat, cattle, consumer product safety, Germany, legislation, food, meat products.

2. Abbott, A. Mad-cow outbreak spurs German drive to combat prion diseases. Nature. 2001 Aug 9. 412(6847): 571-572 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, Germany, strategies to combat the disease, prion diseases, protective measures.

3. Adam, D. Fears rise over BSE infection in UK abattoirs. Nature. 2001 Jun 14. 411(6839): 728 ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: Bovine spongiform encephalopathy, slaughter plants, transmission of contaminated material, UK.

4. Adam, D. Catgut sutures--possible BSE risk. Australian Veterinary Journal. 2001 Apr. 79(4): 245-246 ISSN: 0005-0423
   NAL call no. 41.8 Au72
   Descriptors: Bovine spongiform encephalopathy, catgut, transmission, adverse effects, standards, cattle, risk factors.

5. Adam, D. Review blames BSE outbreak on calf feed. Nature. 2001 Aug 2. 412(6846): 467. ISSN: 0028-0836
   NAL call no. 472 N21
   Descriptors: BSE, calf feeding

6. Aguzzi A. Bovins, humains: quelles connexions? [Cattle and people: what are the connections?] Biofutur. 2001, No. Hors Serie, 26-29; 11 ref. ISSN: 0294-3506. In French.
   NAL call no. TP248.13 B565
   Descriptors: asymptomatic infections, bovine spongiform encephalopathy, brain, carrier state, cattle diseases, NvCreutzfeldt-Jakob disease, digestive tract, routes of disease transmission, lymphocytes, prion diseases.

7. Aguzzi, A.; Montrasio, F.; Kaeser, P.S. Prions: Health scare and biological challenge. Nature Reviews: Molecular Cell Biology, 2001 vol. 2, no. 2, pp. 118-126 ISSN: 1471-0072
   Descriptors: yeasts, central nervous system, prion proteins, NvCreutzfeldt-Jakob-disease, BSE, bovine spongiform encephalopathy, cattle, immune system lymphocytes and follicular dendritic cells, transport of prions.

8. Andersson I.; Svendsen L.S.; Gustafsson B. Vad hander med lantbrukets husdjur i katastrofsituationer? [What happens to farm animals in emergency situations?] Svensk Veterinartidning. 2001, 53: 6, 333-339; 11 ref. ISSN: 0346-2250. In Swedish.
   NAL call no. 41.9 SV23
   Descriptors: animal welfare, bovine spongiform encephalopathy, emergencies, foot and mouth disease, livestock.

9. Anil, M.H.; Love, S.; Helps, C.R.; McKinstry, J,L.; Brown, S.N.; Philips, A.; Williams, S.; Shand, A.; Bakirel, T.; Harbour, D. Jugular venous emboli of brain tissue induced in sheep by the use of captive bolt guns. Veterinary Record. 2001, 148: 20, 619-620; 10 ref. ISSN: 0042-4900
   NAL call no. 41.8 V641
   Descriptors: sheep, experimental procedure, jugular sampling, pre- and post-stunning, stunning, electrical stunning, emboli of CNS tissue compared to stunning methods.

10. Axelsson, S. The basic reality of mind and spongiform diseases. Medical Hypotheses. November, 2001; 57 (5): 549-554. ISSN: 0306-9877
    Descriptors: prions, Creutzfeldt-Jakob Disease, bovine spongiform encephalopathy, Alzheimer's disease, chirality, cholinergic system, pathophysiology, beef products.

11. Bachmann, M.F. Complements for mad-cow disease. Trends in Immunology 2001 Jun. 22(6): 297 ISSN: 1471-4906
    NAL call no. QR180 I56
    Descriptors: Bovine spongiform encephalopathy, immunology, complement immunology, cattle.

12. Balter, M. Infectious diseases. Is BSE in sheep a no-brainer? Science. 2001 Oct 26. 294(5543): 771
    NAL call no. 470 Sci2
    Descriptors: BSE, scrapie, cattle, sheep, transmissible spongiform encephalopathies, prion typing.

13. Balter, M. Infectious diseases. Uncertainties plague projections of vCJD toll. Science. 2001 Oct 26. 294(5543): 770-771 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, variant Creutzfeldt-Jakob Disease, potential incidence.

14. Balter, M. Origins of BSE: Intriguing clues to a scrapie-mad cow link Science (US) 2001 vol. 292, no. 5518, pp. 827-829 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, feeds formulation, transmissibility, cattle, sheep, scrapie, prion protein, NvCreutzfeldt-Jakob disease, Uk, France, connection between BSE and NvCJD

15. Baron Thierry, G M; Biacabe, Anne-Gaelle. Molecular analysis of the abnormal prion protein during coinfection of mice by bovine spongiform encephalopathy and a scrapie agent. Journal of Virology. Jan. 2001. v. 75 (1) p. 107-114.
    Abstract: Molecular features of the proteinase K-resistant prion protein (PrP res) may discriminate among prion strains, and a specific signature could be found during infection by the infectious agent causing bovine spongiform encephalopathy (BSE). To investigate the molecular basis of BSE adaptation and selection, we established a model of coinfection of mice by both BSE and a sheep scrapie strain (C506M3). We now show that the PrP res features in these mice, characterized by glycoform ratios and electrophoretic mobilities, may be undistinguishable from those found in mice infected with scrapie only, including when mice were inoculated by both strains at the same time and by the same intracerebral inoculation route. Western blot analysis using different antibodies against sequences near the putative N-terminal end of PrP res also demonstrated differences in the main proteinase K cleavage sites between mice showing either the BSE or scrapie PrP res profile. These results, which may be linked to higher levels of PrP res associated with infection by scrapie, were similar following a challenge by a higher dose of the BSE agent during coinfection by both strains intracerebrally. Whereas PrP res extraction methods used allowed us to distinguish type 1 and type 2 PrP res, differing, like BSE and scrapie, by their electrophoretic mobilities, in the same brain region of some patients with Creutzfeldt-Jakob disease, analysis of in vitro mixtures of BSE and scrapie brain homogenates did not allow us to distinguish BSE and scrapie PrP res. These results suggest that the BSE agent, the origin of which remains unknown so far but which may have arisen from a sheep scrapie agent, may be hidden by a scrapie strain during attempts to identify it by molecular studies and following transmission of the disease in mice.
    NAL call no. QR360 J6
    Descriptors: mouse model, scrapie, bovine spongiform encephalopathy, laboratory inoculation, variant differences, identification of strains. proteinase K-resistant prion protein.

16. Bartels, Dennis. Mad cows and market forces. Journal of Human Ecology. May, 2001; 12 (3): 163-170. ISSN: 0970-9274
    Descriptors: bovine spongiform encephalopathy, safe of food, consumer attitudes, British government claims.

17. Beale, A. J. BSE and vCJD: what is the future? Journal of the Royal Society of Medicine 2001 May. 94(5): 207-209 ISSN: 0141-0768
    NAL call no. 448.9 R814
    Descriptors: bovine spongiform encephalopathy, Cruetzfeldt-Jakob Disease, epidemiology, bovine based products.

18. The beef CMO and BSE -- making a bad problem worse. Agra Europe (Brit. edition.) 2001, No. 1937, A-1-A-2. ISSN: 0002-1024
    NAL call no. 286.8 AG3AE
    Descriptors: BSE, agricultural crises, EU beef industry, bovine spongiform encephalopathy, consumption, costs, impacts on international trade, subsidies, surpluses, consumer concerns and declining consumption trends.

19. Beiglbock, C. Die Chronic Wasting Disease (CWD) der Cerviden in Nordamerika -- eine Literaturubersicht. [Chronic Wasting Disease (CWD) in cervids in North America -- a review.] Wiener Tierarztliche Monatsschrift. 2001, 88: 6, 147-152; 30 ref. ISSN: 0043-535X. In German with an English summary.
    NAL call no. 41.8 T345
    Descriptors: etiology, diagnosis, disease control, prevention strategies, epidemiology, pathology, reviews, transmissible spongiform encephalopathy; wasting disease, deer, elk, North America.

20. Belay, E. D.; Gambetti, P.; Schonberger, L. B.; Parchi, P.; Lyon, D.R.; Capellari, S.; McQuiston, J.H.; Bradley, K.; Dowdle, G.; Crutcher, J.M.; Nichols, C. R. Creutzfeldt-Jakob disease in unusually young patients who consumed venison. Archives of Neurology. 2001 Oct. 58(10): 1673-1678 ISSN: 0003-9942
    Abstract: BACKGROUND: Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD. OBJECTIVE: To examine the possible transmission of CWD to humans. PATIENTS: Three unusually young patients (aged 28, 28, and 30 years) with CJD in the United States during 1997-2000. METHODS: We reviewed medical records and interviewed family members and state wildlife and agriculture officials. Brain tissue samples were tested using histopathologic, immunohistochemical, immunoblot, or prion protein gene analyses. MAIN OUTCOME MEASURES: Presence or absence of established CJD risk factors, deer and elk hunting in CWD-endemic areas, and comparison of the evidence for the 3 patients with that of a zoonotic link between new variant CJD and bovine spongiform encephalopathy. RESULTS: None of the patients had established CJD risk factors or a history of travel to Europe. Two patients hunted game animals and 1 was a daughter of a hunter. Unlike patients with new variant CJD, the 3 patients did not have a unique neuropathologic manifestation, clinicopathologic homogeneity, uniformity in the codon 129 of the prion protein gene, or prion characteristics different from those of classic variants. CONCLUSIONS: Although the occurrence of 3 unusually young patients with CJD who consumed venison suggested a possible relationship with CWD, our follow-up investigation found no strong evidence for a causal link. Ongoing CJD surveillance remains important for continuing to assess the risk, if any, of CWD transmission to humans.
    Descriptors: chronic wasting disease, zoonotic transmission concerns, case study, NvCreutzfeldt-Jakob disease infection, deer and elk meat, 3 young patients, codon 129.

21. Bergmann, W.; Beringer, H. Kupfermangel, ein moglicher BSE-auslosender Faktor? [Copper deficiency -- a potential factor in BSE?] Journal of Plant Nutrition and Soil Science. 2001, 164: 2, 233-235; 13 ref. ISSN: 1436-8730. In German with an English summary.
    NAL call no. QK867 J68
    Descriptors: BSE, normal and pathogenic prions chemistry, bovine spongiform encephalopathy, copper, trace minerals, mineral deficiencies, reviews.

22. Berthelin, Baker C.; Konold, T.; Clifford, D.; Ryder, S.; Bellworthy, S.; Dexter, G.; Brittin, D.; Wood, D.; Simmonds, M.; Bone, G.; Jeffrey, M. Interim observations on the clinical features of Bovine Spongiform Encephalopathy in sheep of the Romney and Suffolk breeds. Research in Veterinary Science. April, 2001; 70 (Supplement A): 28. ISSN: 0034-5288. 55th Annual Conference on Current Topics in Veterinary Science, Scarborough, England, UK, April 09-12, 2001
    NAL call no. 41.8 R312
    Descriptors: BSE, sheep breed susceptibility differences, transmissible spongiform encephalopathies.

23. Bin Kingombe, C.I.; Luthi, E; Schlosser, H; Howald, D; Kuhn, M; Jemmi, T. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat science. Oxford: Elsevier Science Limited. Jan. 2001. v. 57 (1) p. 35-41. Includes references.
    NAL call no. TX373.M4
    Descriptors: polymerase chain reaction, fish meal, pork, ELISA, monitoring, screening, mitochondrial DNA, cytochrome B, genes, cattle, prevention, bovine spongiform encephalopathy.

24. Bindon, B.M.; Jones, N.M. I. Cattle supply, production systems and markets for Australian beef. Australian Journal of Experimental Agriculture. 2001; 41 (7): 861-877. ISSN: 0816-1089
    NAL call no. 23 Au792
    Descriptors: beef cattle, market impacts, BSE, foot and mouth disease, history of industry, 20^th century, brucellosis, tuberculosis, Dick Austen, trade, standards, food safety concerns, new market forces, Australia.

25. Birkett, C.R.; Hennion, R.M.; Bembridge, D.A.; Clarke, M.C.; Chree, A.; Bruce, M.E.; Bostock, C. J. Scrapie strains maintain biological phenotypes on propagation in a cell line in culture. EMBO Journal 2001 Jul 2. 20(13): 3351-3358. ISSN: 0261-4189
    Abstract: Bovine spongiform encephalopathy (BSE) and its human equivalent, variant Creutzfeldt-Jakob disease (vCJD), are caused by the same strain of infectious agent, which is similar to, but distinct from, >20 strains of their sheep scrapie homologue. A better understanding of the molecular strain determinants could be obtained from cells in monoculture than from whole animal studies where different cell targeting is commonly a strain-related feature. Although a few cell types can be infected with different strains, the phenotypes of the emergent strains have not been studied. We have cured the scrapie-infected, clonal SMB cell line with pentosan sulfate, stably re-infected it with a different strain of scrapie and shown that biological properties and prion protein profiles characteristic of each original strain are propagated faithfully in this single non-neuronal cell type. These findings attest to the fact that scrapie strain determinants are stable and host-independent in isolated cells.
    NAL call no. QH506 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, molecular differences, strains, sheep scrapie, clonal, SMB.

26. Bittante, G. Mercato e tecnica di alimentazione: cosa cambia dopo la BSE. [The market and animal feeding techniques: changes since the BSE crisis.] Informatore Agrario. 2001, 57: 11, 81-85. ISSN: 0020-0689. In Italian.
    NAL call no. 281.8 IN32
    Descriptors: animal feeding, beef, bovine spongiform encephalopathy, disease control, economic impact; feeds, producer prices.

27. Blood-based prion test. Analytical Chemistry. 2001 May 1. 73(9): 252A ISSN: 0003-2700
    NAL call no. 381 J825A
    Descriptors: diagnostic test, screening live mammals, fluorescently labeled peptide from PrP and peptide antibodies, economical preclinical method, CE or size exclusion HPLC.

28. Bol, P. BSE en andere prionziekten. [BSE and other prion diseases] Nederlands Tijdschrift voor Tandheelkunde 2001 Feb. 108(2): 72-73 ISSN: 0028-2200 In Dutch.
    Descriptors: transmissible spongiform encephalopathies.

29. Bonnardiere, C la; la Bonnardiere, C. BSE: le depistage s'organise. [Early detection of bovine spongiform encephalopathy is progressing.] Biofutur. 2001, No. Hors Serie, 52-56; 8 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: bovine spongiform encephalopathy, diagnostic techniques, Western blot assay, Prionics, brain stem testing. Biorad enzyme assay.

30. Bosch, X. BSE panic spreads to Spain. Nature Medicine. 2001 Feb. 7(2): 138 ISSN: 1078-8956
    Descriptors: bovine spongiform encephalopathy, incidence, disease risks, Spain.

31. Bosch, X. European concern over BSE transmission. JAMA. 2001 Jan 24-31. 285(4): 397-398. ISSN: 0098-7484
    NAL call no. 448.9 AM37
    Descriptors: bovine spongiform encephalopathy, transmissibility, human and animal health risks.

32. Bradley, R.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Bovine spongiform encephalopathy and its relationship to the new variant form of Creutzfeldt-Jakob disease. Prions: a challenge for science, medicine and public health-system. 2001, 105-144; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
    NAL call no. QR1 C66 v. 7
    Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, prion diseases, pathogenesis

33. Bradley, R. A brief overview of bovine spongiform encephalopathy and related diseases including a TSE risk analysis of bovine starting materials used during the manufacture of vaccines for use in humans. Przegl Epidemiol. 2001. 55(3): 387-405 ISSN: 0033-2100
    Descriptors: BSE, transmissible spongiform encephalopathies, bovine based pharmaceutical products, vaccines, public health risks

34. Bradley, R. Obecna sytuacja badan nad pasozowalnymi encefalopatiami gabczastymi. [The current situation of investigation of bovine spongiform encephalopathy] Przeglad Epidemiologiczny. 2001. 55(1 Suppl 2): 37-59 ISSN: 0033-2100. In Polish.
    Descriptors: BSE, Polish situation, epidemiology.

35. Bratberg B.; Benestad S.L.; Schonheit J. "Ny type" scrapie. ["New type" scrapie.] Norsk Veterinaertidsskrift. 2001. 113: 2, 79-80; 4 ref. ISSN: 0332-5741. In Norwegian.
    NAL call no. 41.8 N81
    Descriptors: BSE, bovine spongiform encephalopathy; pathology; scrapie, experimental animals.

36. Brazil, Instituto Brasileiro de Economia. Vaca louca. [Mad cow.] Agroanalysis. 2001, 21: 2, 39-46. ISSN: 0100-4298. In Portuguese.
    Descriptors: BSE, cattle prion diseases, bovine spongiform encephalopathy, pastures grazing systems, cattle feeding, low risk.

37. Bren, L. Trying to keep "Mad Cow Disease" out of U.S. herds. FDA Consumer Rockville, Md. : Food and Drug Administration, Department of Health & Human Services. Mar/Apr 2001. v. 35 (2) p. 12-14. ISSN: 0362-1332
    NAL call no. HD9000.9.U5A1
    Descriptors: bovine spongiform encephalopathy, disease prevention, Creutzfeldt-Jakob Disease. USDA, federal government, disease transmission, surveillance, meat inspection, Federal regulations, meat and livestock industry, ruminant feeding, consumer protection, USA.

38. Brewer, M.S. Bovine spongiform encephalopathy--food safety implications. Adv Food Nutr Res. San Diego : Academic Press, c1989-. 2001. v. 43 p. 265-317. ISSN: 1043-4526
    NAL call no. TX537.A38
    Descriptors: bovine spongiform encephalopathy, food safety, food contamination, rendering, disease transmission, prions, strains, prion diseases, genetic factors, species differences, human health risks, Creutzfeldt-Jakob-Disease, infectivity, bovine-based products, tallow, gelatin, diagnostic techniques, surveillance, disease control, disease prevention, literature reviews, UK, USA.

39. Bromley, D.W. Mad cows, drugged cows, and juggled genes: purpose and necessity in science policy and public opinion. Choices. The Magazine of Food, Farm, and Resources Issues. 2001, No. 2, 6-9. ISSN: 0886-5558
    NAL call no. HD1751.C45
    Descriptors: beef, biosafety, BSE, bovine spongiform encephalopathy, food contamination, food safety, milk, food related public health scares, public, BST.

40. Brown, D.R. BSE: a post-industrial disease? Chemistry and Industry. 2001, No. 3, 73-76; 13 ref. ISSN: 0009-3068
    Descriptors: BSE, etiology, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob disease, prion diseases, cattle, human health risks.

41. Brown, D.R. BSE did not cause variant CJD: An alternative cause related to post-industrial environmental contamination. Medical Hypotheses. November, 2001; 57 (5): 555-560. ISSN: 0306-9877
    Descriptors: prion diseases, Bovine spongiform encephalopathies, NvCreutzfeldt-Jakob Disease, etiology, theories, manganese-rich pollutants.

42. Brown, Paul. Afterthoughts about bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. Emerging Infectious Diseases. 2001; 7 (3 Supplement): 598-600. ISSN: 1080-6040.
    NAL call no. RA648.5 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, issues, new diseases, prion diseases

43. Brown, P. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease. BMJ Clinical Research ed. 2001 Apr 7. 322(7290): 841-844 ISSN: 0959-8138
    Descriptors: Creutzfeldt-Jakob Syndrome, etiology, epidemiology, age distribution, cattle.

44. Brown, P.; Will, R.G.; Bradley, R.; Asher, D.M.; Detwiler, L. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: background, evolution, and current concerns. Emerging Infectious Diseases 2001 Jan-Feb. 7(1): 6-16 ISSN: 1080-6040
    Abstract: The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom, which began in 1986 and has affected nearly 200,000 cattle, is waning to a conclusion, but leaves in its wake an outbreak of human Creutzfeldt-Jakob disease, most probably resulting from the consumption of beef products contaminated by central nervous system tissue. Although averaging only 10-15 cases a year since its first appearance in 1994, its future magnitude and geographic distribution (in countries that have imported infected British cattle or cattle products, or have endogenous BSE) cannot yet be predicted. The possibility that large numbers of apparently healthy persons might be incubating the disease raises concerns about iatrogenic transmissions through instrumentation (surgery and medical diagnostic procedures) and blood and organ donations. Government agencies in many countries continue to implement new measures to minimize this risk.
    NAL call no. RA648.5 E46
    Descriptors: BSE, NvCreutzfeldt-Jakob Disease, UK, human disease risks, tranmissibility concerns, incidence, iatrogenic transmission risks, governmental measures, risk control, epidemiology.

45. Brown, P. The pathogenesis of transmissible spongiform encephalopathy: routes to the brain and the erection of therapeutic barricades. Cellular and Molecular Life Sciences. 2001, 58: 2, 259-265; 51 ref. ISSN: 1420-682X
    NAL call no. QH301 C45
    Descriptors: BSE, bovine spongiform encephalopathy, pathogenesis, NvCreutzfeldt-Jakob disease, treatments, prion diseases, scrapie, spongiform encephalopathy.

46. Brugere-Picoux, J.; Brugere, H. Encephalopathie spongiforme bovine: un tournant dans l'evolution de l'epidemie? [Bovine spongiform encephalopathy: a change in the etiology of the epidemic?] Revue de Medecine Interne, 2001 Aug. 22(8): 693-698 ISSN: 0248-8663. In French.
    Descriptors: BSE, etiology, disease incidence.

47. BSE bij gezelschapsdieren. [BSE in companion animals] Tijdschrift voor Diergeneeskunde 2001 Feb 15. 126(4): 120-121 ISSN: 0040-7453. In Dutch.
    NAL call no. 41.8 T431
    Descriptors: BSE, issues of transmissible spongiform encephalopathies, cats, dogs.

48. Butler, D. Spotlight on scrapie in hunt for sheep BSE. Nature. 2001 Nov 1. 414(6859): 7 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: Bovine spongiform encephalopathy, diagnosis, scrapie, sheep, cattle, Great Britain.

49. Buzby, J.C.; Detwiler, L.R. BSE: anatomy of a crisis. Choices. The Magazine of Food, Farm, and Resources Issues. 2001, No.2, 41-45. ISSN: 0886-5558
    NAL call no. HD1751.C45
    Descriptors: agricultural crises, BSE, bovine spongiform encephalopathy, Creutzfeldt NvJakob disease, economic impact, epidemics, impacts on exports and international trade, meat and livestock industry, US policies, cattle protection.

50. Calavas, D.; Ducrot, C.; Baron, T.; Morignat, E.; Vinard, J.L.; Biacabe, A.G.; Madec, J.Y.; Bencsik, A.; Debeer, S.; Eliazsewicz, M. Prevalence of BSE in western France by screening cattle at risk: preliminary results of a pilot study. Vet Rec. London : The British Veterinary Association. July 14, 2001. v. 149 (2) p. 55-56. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: cattle, bovine spongiform encephalopathy, disease prevalence, age risk, France

51. Calza, L.; Manfredi, R.; Chiodo, F. Epidemia di encefalopatia spongiforme bovina e nuova variante della malattia di Creutzfeldt-Jakob nell'uomo. Ultime acquisizioni sulle malattie da prioni. [Epidemics of bovine spongiform encephalopathy and new variant of Creutzfeldt-Jakob disease in humans. Most recent findings on prion disease.] Recenti Progressi in Medicina. 2001 Feb. 92(2): 140-149 ISSN: 0034-1193. In Italian.
    Abstract: Prion diseases have been popularized by extensive media coverage of bovine spongiform encephalopathy (BSE) or "mad cow disease" epidemic, observed in Great Britain since 1986, and new variant Creutzfeldt-Jakob disease (nvCJD), reported for the first time in 1996. In contrast to the classical form of the disease, nvCJD affects younger patients, presents a relatively longer duration of illness and is caused by the same agent as BSE. Evidence from laboratory studies now strongly supports the hypothesis that new variant represents human form of animal disease, linked to exposure, probably through food, to bovine prions. Number of BSE reports in the United Kingdom began to decline in 1993, and has continuously decreased year by year since then, but a great worry spread in European countries in association with new BSE reported cases outside of the Great Britain, and increasing incidence of nvCJD. New epidemiological, clinical, histopathological and experimental data on prion diseases are reviewed, focusing our attention on the possible transmission of prion proteins from animals to humans.
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, epidemiology, clinical data, UK, European incidence, transmission, human health risks.

52. Caramelli, M; Ru, G; Casalone, C; Bozzetta, E; Acutis, PL; Calella, A; Forloni, G. Evidence for the transmission of scrapie to sheep and goats from a vaccine against Mycoplasma agalactiae. Veterinary Record. 2001, 148: 17, 531-536; 21 ref. ISSN: 0042-4900
    NAL call no. 41.8 V641
    Descriptors: BSE, bovine spongiform encephalopathy, brain, codon 171, cross infection, epidemiology, genotypes, mortality, polymorphism, scrapie, transmission, Mycoplasma agalactiae vaccine as infective source, sheep, goats, brain lesions.

53. Cardone, F.; Pocchiari, M. A role for complement in transmissible spongiform encephalopathies. Nature Medicine 2001 Apr. 7(4): 410-411 ISSN: 1078-8956
    Descriptors: BSE, CJD, blood factors, role in disease formation.

54. Carlier, F. Vers une economie du principe de precaution. [Economy of the precautionary principle.] Biofutur. 2001, No. Hors Serie, 16-20; 7 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, traceability measures, bovine spongiform encephalopathy, consumer protection, disease prevention, labeling, meat and meat products, prion diseases, Europe.

55. Carson, C.; McKay, J.S.; Brooks, H.W.; Kelly, D.F.; Stidworthy, M.F.; Wibbelt, G.; Morgan, K.L. Establishment and maintenance of a longitudinal study of bovine spongiform encephalopathy (the ULiSES scheme). Preventive Veterinary Medicine. 2001 Oct 11. 51(3-4): 245-257 ISSN: 0167-5877
    Abstract: This paper addresses the issues of tracing and compliance encountered in setting up and maintaining a UK-wide 5-year observational study of beef cattle. The 5-year prospective study was initiated in 1997 to investigate the occurrence of bovine spongiform encephalopathy (BSE) in a single herd of pedigree Aberdeen Angus cattle, in which BSE had been detected at low prevalence. The study was given the acronym ULiSES (University of Liverpool Spongiform Encephalopathy Scheme). All cattle present on the farm at the start of the scheme were registered as members of the study population (n=320), as were all calves standard histopathological techniques) for the presence of spongiform change. Remaining samples were stored at -70 degrees C for future investigation by alternative tests. At the halfway point of the scheme in October 1999, 75.2% (506/673) of the study population was still alive; 42% (284) of the population was still alive on the study farm and 33% (222) was distributed on other farms throughout the UK. Complete sets of specimens had been recovered from 77% (129/167) of dead animals. All brainstem sections were negative by histopathological examination. No suspect cases of BSE were reported in ULiSES animals. Failure to recover specimens occurred principally in animals which had left the study farm. The main cause of specimen loss was a failure of compliance in a small number of individuals who had purchased large numbers of ULiSES animals, and subsequently slaughtered them without contacting the University. Despite this, farmer compliance was generally high. The ULiSES scheme shows the feasibility of a country-wide longitudinal observational study spanning a period of several years and indicates the large impact of small numbers of non-compliant individuals.
    NAL call no. SF601.P7
    Descriptors: University of Liverpool Spongiform Encephalopathy Scheme, Aberdeen Angus beef cattle, 5 year observational study, post mortem sampling, BSE surveillance.

56. Cashman, N.R. Transmissible spongiform encephalopathies: Vaccine issues. In Developments in Biologicals. Evolving scientific and regulatory perspectives on cell substrates for vaccine development. 2001; (106): 455-461. Fred Brown; Andrew Lewis Jr.; Keith Peden; and Philip Krause Eds S. Karger Publishers Inc., 79 Fifth Avenue, New York, NY, 10003, USA; S. Karger AG, CH 4009, Basel, Switzerland. ISBN 1424-6074
    NAL call no. QR180.3 D4 v. 106
    Descriptors: BSE, transmissible spongiform encephalopathies, vaccine development, techniques, approaches.

57. Caughey, B.; Dobson, C. M. (ed.); Ellis, R. J. (ed.); Fersht, A. R. Prion protein inter-conversions. Protein misfolding and disease. Papers of a Discussion Meeting held at the Royal Society, London, UK, on 23 and 24 February 2000. Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 2001, 356: 1406, 197-202; Many ref. ISSN: 0962-8436.
    NAL call no. 501 L84Pb
    Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt Jakob disease, in vitro models, prion diseases, conversion inhibitors, prion proteins, scrapie

58. Cervenakova, L. The safety of human blood: experimental TSE/prion infectivity studies. Transfusion Clinique et Biologique Journal de la Societe Francaise de Transfusion Sanguine. 2001 Jun. 8(3): 260. ISSN: 1246-7820
    Descriptors: Cattle, blood transfusion, adverse effects, diseases, Creutzfeldt-Jakob Syndrome, transmission, prevention and control, bovine spongiform encephalopathy, Great Britain, human, prion diseases, safety.

59. Cesar, Isigidi K.; Luthi, E.; Schlosser, H.; Howald, D.; Kuhn, M.; Jemmi, T. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat Science. 2001, 57: 1, 35-41; 17 ref. ISSN: 0309-1740
    NAL call no. TX373 M4
    Descriptors: PCR- based assay, screening method, animal meals, bovine spongiform encephalopathy, disease prevention, heat treated meat meal, meat products, pork, polymerase chain reaction.

60. Cesbron, JY; Lemaire, C; Gagnon, J. Comment se propage l'infection. [How the infection is propagated.] Biofutur. 2001, No. Hors Serie, 37-40; 8 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, bovine spongiform encephalopathy, mouse scrapie, disease course and models, ileum, immune system, laboratory animals, peripheral nerves, scrapie, comparison.

61. Clarke, A.R.; Jackson, G.S.; Collinge, J.; Dobson, C.M. (ed.); Ellis, R.J. (ed.); Fersht, A.R. The molecular biology of prion propagation. Protein misfolding and disease. Papers of a Discussion Meeting held at the Royal Society, London, UK, on 23 and 24 February 2000. Philosophical Transactions of the Royal Society of London. Series B,-Biological Sciences. 2001, 356: 1406, 185-195; Many ref. ISSN: 0962-8436
    NAL call no. 501 L84Pb
    Descriptors: BSE, bovine spongiform encephalopathy; NvCreutzfeldt Jakob disease; prion protein molecular biology, scrapie, PrPSc, prion propagation.

62. Coe, John E.; Race, Richard E.; Ross, Mary J. Serological evidence for an inflammatory response in murine scrapie. Journal of Infectious Diseases. 15 January, 2001; 183 (2): 185-191. ISSN: 0022-1899.
    NAL call no. 448.8 J821
    Descriptors: transmissible spongiform encephalopathies, murine scrapie experimental model, C57BL10 and IRW mice, serum amyloid P component levels, possible diagnostic test.

63. Collinge, J. Prion diseases of humans and animals: their causes and molecular basis. Annual Review of Neuroscience. 2001. 24: 519-550 ISSN: 0147-006X
    Abstract: Prion diseases are transmissible neurodegenerative conditions that include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) and scrapie in animals. Prions appear to be composed principally or entirely of abnormal isoforms of a host-encoded glycoprotein, prion protein. Prion propagation involves recruitment of host cellular prion protein, composed primarily of alpha-helical structure, into a disease specific isoform rich in beta-sheet structure. The existence of multiple prion strains has been difficult to explain in terms of a protein-only infections agent, but recent studies suggest that strain specific phenotypes can be encoded by different prion protein conformations and glycosylation patterns. The ability of a protein to encode phenotypic information has important biological implications. The appearance of a novel human prion disease, variant CJD, and the clear experimental evidence that it is caused by exposure to BSE has highlighted the need to understand the molecular basis of prion propagation, pathogenesis, and the barriers limiting intermammalian transmission. It is unclear if a large epidemic of variant CJD will occur in the years ahead.
    NAL call no. QP351.A68
    Descriptors: NvCreutzfeldt-Jakob disease, bovine spongiform encephalopathy, scrapie, abnormal isoforms, prion protein isoforms, prion propagation, pathogenesis, barriers to transmission.

64. Comincini, S.; Foti, M.G.; Tranulis, M.A.; Hills, D.; Di Guardo, G.; Vaccari, G.; Williams, J.L.; Harbitz, I.; Ferretti, L. Genomic organization, comparative analysis, and genetic polymorphisms of the bovine and ovine prion Doppel genes (PRND). Mammalian Genome. 2001 Sep. 12(9): 729-733 ISSN: 0938-8990
    Abstract: The doppel protein (Dpl) is a prion-like protein encoded by the gene PRND, which has been found downstream of the prion gene, PRNP, in human and mouse. This paper describes the isolation and structural organization of the bovine and ovine PRND genes, which are composed of two exons compared with the three of human and mouse. Intergenic distances between PRNP and PRND were covered by means of long-range PCR and found to be 16.8 and 20 kb, in cattle and sheep respectively. The 5' and 3' untranslated regions (UTR) were analyzed to identify transcription regulatory sequences and compared with those from the PRND and PRNP sequences published for other species. Three polymorphisms (R50H, N110H, and R132Q) were revealed in the cattle coding region; two synonymous substitutions (I12I, A26A) were found in sheep. None of the polymorphisms was significantly associated with either Bovine Spongiform Encephalopathy (BSE) in cattle or scrapie in sheep.
    NAL call no. QL738.5.M359
    Descriptors: doppel protein, bovine, ovine PRND genes, structure, isolation, PCR polymorphisms, association with BSE and scrapie.

65. Comincini, S.; Castiglioni, B. M.; Foti, G. M.; Del Vecchio, I.; Ferretti, L. Isolation and molecular characterization of rasfadin, a novel gene in the vicinity of the bovine prion gene. Mammalian Genome. 2001 Feb. 12(2): 150-156 ISSN: 0938-8990
    Abstract: A novel gene, rasfadin (RASSF2) was identified close to the bovine prion gene, and its genomic structure was derived with a combination of exon trapping and RACE. The gene covers at least 28 kb and maps to the same chromosomal region as the prion gene in cattle, sheep, and human. The RASSF2 ORF is composed of 987 base pairs divided into nine exons and shows a high nucleotide (88%) and amino acid similarity (95%) with a previously described human cDNA, KIAA0168. The bovine 3'UTR region is significantly shorter than the human counterpart, but shares with it two highly conserved nucleotide blocks. The expression of the gene was investigated in brain, liver, and spleen. Alternative splicing yields a shorter product in the liver composed of only four exons. Computer analysis showed a highly significant similarity of the rasfadin protein with the Ras association (Ral-GDS/AF-6) domain family 2 and with the afadin family, respectively, for the longer brain/spleen and the shorter liver variants.
    NAL call no. QL738.5.M359
    Descriptors: rasfadin gene, bovine prion gene, exon trapping, RACE, cattle, sheep, humans

66. Cooley, W.A.; Clark, J.K.; Ryder, S.J.; Davis, L.A.; Farrelly, S.S.J.; Stack, M.J. Evaluation of a rapid western immunoblotting procedure for the diagnosis of bovine spongiform encephalopathy (BSE) in the UK. J Comp Pathol. London : W.B. Saunders Company Ltd. July 2001. v. 125 (1) p. 64-70.
    Abstract: Bovine brain tissue samples from 625 UK cattle, clinically suspected as bovine spongiform encephalopathy (BSE) cases, were used in a blind analysis to assess a rapid Western immunoblotting technique (Prionics Check; Prionics AG, Zurich), which detects bovine disease-specific protease-resistant prion protein (PrP(Sc)). By means of statutory histopathological examination, 599 of the 625 cattle were confirmed as BSE cases by the demonstration of spongiform encephalopathy, the remaining 26 being classified as negative. Duplicate samples from the same animals were also examined by electron microscopy for the presence of abnormal brain fibrils (scrapie-associated fibrils; SAFs). The Prionics technique showed a high sensitivity, particularly when compared with the fibril detection test; the detection rates were 99.3% and 92.0% respectively, with histopathology being used as the "gold standard". The false negative results by the Prionics test were possibly related to the sampling procedure. Analysis of 50 BSE-positive samples revealed similar glycoprofiles, the majority of PrP(Sc)isoforms being di-glycosylated protein. The Prionics test also detected PrP(Sc)in the four brain samples from the 26 histopathologically negative animals, apparently reducing the specificity of the test to 84.6%; however, confirmatory positive results in these samples were obtained by demonstrating SAF or by immunohistochemical examination, or both. It was concluded that the Prionics test detected PrP(Sc)in a small percentage (0.64%) of clinically suspected BSE cases showing no spongiform change. Since January 2000, the Prionics Western blot test has been introduced as one of the statutory tests for the diagnosis of clinically suspected BSE and scrapie cases in the UK.
    NAL call no. 41.8 J82
    Descriptors: cattle, bovine spongiform encephalopathy, immunoblotting, brain, diagnostic techniques and value, histopathology, electron microscopy, prion proteins, UK.

67. Corn, Joseph L.; Nettles, Victor F. Health protocol for translocation of free-ranging elk. Journal of Wildlife Diseases 37(3), July, 2001: 413-426.
    NAL call no. 41.9 W64B
    Descriptors: elk, Cervus elaphus, health protocol for restoration programs, health scoring, ectoparasites, some diseases, internal parasites, quarantines, diagnostic testing, prophylactic treatment.

68. Coulthart, M.B.; Cashman, N.R. Variant Creutzfeldt-Jakob disease: a summary of current scientific knowledge in relation to public health. CMAJ-Canadian Medical Association Journal 2001 Jul 10; 165(1): 51-58. ISSN: 0820-3946
    Abstract: The prion diseases pose unique scientific, medical, veterinary and regulatory challenges. Here, we summarize current information bearing on the natural history, pathobiology and epidemiology of these disorders and public policy responses to the potential threats to public health posed, particularly, by bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease (vCJD). Six years after the first case reports of vCJD, there is still no clear indication of the magnitude of the primary epidemic, or of the likelihood of lateral transmission of this untreatable disease by iatrogenic means, particularly by blood and blood products. However, the unsettling nature of the available evidence warrants prudence regarding public health policy and regulation, as well as a forward-looking approach to research.
    NAL call no. R11 C3
    Descriptors: NvCJD, natural history, pathobiology, epidemiology, public health risks, UK, transmission issues, iatrogenic transfer.

69. Cousens, S.; Smith, P.G.; Ward, H.; Everington, D.; Knight, R.S.; Zeidler, M.; Stewart, G.; Smith-Bathgate, E.A.; Macleod, M. A.; Mackenzie, J.; Will, R.G. Geographical distribution of variant Creutzfeldt-Jakob disease in Great Britain, 1994-2000. Lancet. 2001 Mar 31. 357(9261): 1002-1007 ISSN: 0140-6736
    Abstract: BACKGROUND: Geographical variation in the distribution of variant Creutzfeldt-Jakob disease (vCJD) might indicate the transmission route of the infectious agent to man. We investigated whether regional incidences of vCJD were correlated with regional dietary data. METHODS: The National CJD Surveillance Unit prospectively identified 84 people with vCJD up to Nov 10, 2000, in Great Britain. Their lifetime residential histories were obtained by interviews with a close relative. Cumulative incidences of vCJD by standard region were calculated. Grid references for places of residence in 1991 were identified and evidence of geographical clusters were sought. Data on diet in the 1980s were analysed for regional correlations with vCJD incidence. The socioeconomic status of the places of residence of people with vCJD was compared with that of the general population. FINDINGS: vCJD incidence was higher in the north of Great Britain than the south. The rate ratio (north vs south) was 1.94 (95% CI 1.27-2.98). The mean Carstairs' deprivation score for areas of residence of people with vCJD was -0.09 (-0.73 to 0.55), which is close to the national average of zero. Regional rates of vCJD were correlated with consumption of other meat or meat products as classified and recorded by the Household Food Consumption and Expenditure Survey (r=0.72), but not with data from the Dietary and Nutritional Survey of British Adults. Five people with vCJD in Leicestershire formed a cluster (p=0.004). INTERPRETATION: Regional differences in vCJD incidence are unlikely to be due to ascertainment bias. We had difficulty determining whether regional variations in diet might cause these differences, since the results of dietary analyses were inconsistent.
    NAL call no. 448.8 L22
    Descriptors: disease incidence, NvCreutzfeldt-Jakob Disease, diet analysis, socioeconomic levels, regional differences, Carstairs’ deprivation score, results.

70. Crise de l'ESB: le secteur bovin toujours dans l'incertitude. [The BSE crisis: still uncertainty for the cattle sector.] Chambres-d'Agriculture. 2001, No. 896, 3-9. ISSN: 0396-7883. In French.
    NAL call no. 14 T69
    Descriptors: BSE, effects on beef consumption and international trade, bovine spongiform encephalopathy, cattle diseases, 2 scenarios, cattle prices, prion diseases, GATT trade agreements

71. Cutlip, R.C.; Miller, J.M.; Hamir, A.N.; Peters, J.; Robinson, M.M.; Jenny, A.L.; Lehmkuhl, H.D.; Taylor, W.D.; Bisplinghoff, F.D. Resistance of cattle to scrapie by the oral route. Canadian Journal of Veterinary Research; Revue Canadienne de Recherche Veterinaire. 2001 Apr. 65(2): 131-132 ISSN: 0830-9000
    Abstract: Early epidemiological information indicated that bovine spongiform encephalopathy (BSE) originated from scrapie in sheep. The question arose if scrapie in North America would induce a BSE-like disease in cattle. Six years ago, we reported that brain tissue from sheep with scrapie caused a neurologic disease when injected directly into the brains of cattle, but the disease induced was different from BSE as it occurs in the United Kingdom and Europe. Here, we report that cattle fed raw brain or meat and bone meal and tallow prepared from sheep with scrapie remained normal for 8 years after exposure. This work indicates that cattle are highly resistant to North American scrapie by the oral route.
    NAL call no. SF601.C24
    Descriptors: BSE, scrapie, cattle, sheep, North American scrapie, oral feeding of raw brain and meat, resistance to infection.

72. Cyranoski, D. Japan's first BSE case fuels fears elsewhere. Nature. 2001 Sep 27. 413(6854): 337 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: BSE, Japan, cattle, introduced zoonosis.

73. de Vries, H. Het afvoeren van alle dieren van een bedrijf naar de destructie bij het aantonen van BSE van een dier op dat bedrijf. Een goede strategie? [Destruction of all animals on the farm after diagnosis of BSE in one animal. Is that a good strategy?] Tijdschrift voor Diergeneeskunde 2001 Feb 15. 126(4): 113-115 ISSN: 0040-7453. In Dutch.
    NAL call no. 41.8 T431
    Descriptors: BSE, bovine spongiform encephalopathies, cattle, livestock, disease control, veterinary care issues, The Netherlands.

74. Dealler, S. Should young UK cattle be considered free of BSE or is it endemic? British Food Journal. 2001, 103: 4, 264-280; 30 ref. ISSN: 0007-070X
    NAL call no. 389.8 B77
    Descriptors: BSE, bovine spongiform encephalopathy; calves; disease transmission; epidemiology; law; pathogenesis; reviews

75. Debeer, S.O.; Baron, T.G. Bensik, A.A. Immunohistochemistry of PrPsc within bovine spongiform encephalopathy brain samples with graded autolysis. Journal of Histochemistry and Cytochemistry 2001 Dec. 49(12): 1519-1524 ISSN: 0022-1554
    Abstract: Bovine spongiform encephalopathy (BSE) is a transmissible neurodegenerative disease of cattle. Clinical diagnosis can be confirmed by investigation of both spongiform changes and abnormal prion protein (PrPsc), a marker considered specific for the disease. Tissue autolysis, often unavoidable in routine field cases, is not compatible with histological examination of the brain even though PrPsc is still detectable by immunoblotting. To determine how autolysis might affect accurate diagnosis using PrPsc immunohistochemistry, we studied 50 field samples of BSE brainstem (obex) with various degrees of autolysis. We demonstrated that the antigen-unmasking pretreatments necessary for PrPsc immunohistochemistry were compatible with the preservation of autolyzed brain sections and that PrPsc detection was unaffected by autolysis, even though anatomic markers were sometimes lost. In tissue samples in which anatomic sites were still recognizable, PrPsc accumulation was detected in specific gray matter nuclei. In samples with advanced autolysis, PrPsc deposits were still observed, at least at the cellular level, as an intraneuronal pattern. We found that the sensitivity of PrPsc immunohistochemistry as a diagnostic method for BSE was undiminished even by severe tissue autolysis.
    NAL call no. 381 J8222
    Descriptors: BSE, diagnosis, brainstem tissue, field samples, method sensitivity.

76. Deslys, J.P.; Lasmezas, C.I.; Comoy, E.; Domont, D. Diagnosis of bovine spongiform encephalopathy. Vet J. London : Balliere Tindall, c1997-. Jan 2001. v. 161 (1) p. 1-3. ISSN: 1090-0233
    NAL call no. SF601.V484
    Descriptors: bovine spongiform encephalopathy, diagnosis, diagnostic techniques.

77. Deslys, Jean Philippe.; Picot, Andre. La vache folle: les risques pour l'homme. [Mad cow disease: the risks for humans.] Dominos (Paris, France); 229. Paris : Flammarion, c2001. 127 p., ill., map. In French. ISBN: 2080300261
    NAL call no. SF967.B63D47 2001
    Descriptors: bovine spongiform encephalopathy, public health risk assessment.

78. Deslys, J. P.; Comoy, E.; Hawkins, S.; Simon, S.; Schimmel, H.; Wells, G.; Grassi, J.; Moynagh, J. Screening slaughtered cattle for BSE. Nature. 2001 Jan 25. 409(6819): 476-478 ISSN: 0028-0836
    NAL call no. 472 N21
    Descriptors: brain, International organizations, slaughter survey, abattoirs, prion protein, bovine spongiform encephalopathy; European Union, cattle.

79. Doherr, M.G.; Heim, D.; Fatzer, R.; Cohen, C.H.; Vandevelde, M.; Zurbriggen, A. Targeted screening of high-risk cattle populations for BSE to augment mandatory reporting of clinical suspects. Prev. Vet. Med. Amsterdam, The Netherlands : Elsevier Science B.V. Sept 20, 2001. v. 51 (1/2) p. 3-16. ISSN: 0167-5877. In the special issue: Structure mirrors function / edited by M. Thrusfield and E. Goodall. Paper presented at a meeting held March 29-31, 2000, Edinburgh, Scotland, UK.
    Abstract: In Switzerland, the first case of bovine spongiform encephalopathy (BSE) was diagnosed in November 1990. Case numbers peaked in 1995, with a total of 352 BSE cases identified by 30 April 2000. Reporting of clinically suspect cattle is currently the most commonly used method world-wide to detect BSE cases. The effectiveness of mandatory reporting depends on a variety of factors; for other diseases passive surveillance underestimates the incidence of clinical cases. The efficiency of passive surveillance systems for BSE will remain unknown until screening tests able to identify clinically affected cattle have been applied in several countries. This paper provides the first detailed description of a targeted screening programme for BSE. Two populations of cows >24 months of age were included in the targeted screening: (i) cows found dead or culled on site where the carcass was submitted to rendering (fallen stock) and (ii) cows with health-related problems unfit for routine slaughter that were slaughtered under emergency procedures (emergency slaughter). Between 1992 and 1999, on average 81 clinical BSE suspects per year were reported to the veterinary authorities (passive surveillance), of which 43% were confirmed with BSE. A total of 30 clinical cases were captured by passive surveillance and an additional 20 BSE cases detected by targeted screening between May 1999 and April 2000. The odds of finding a BSE case was 49 times higher in the fallen stock and 58 times higher in emergency-slaughtered cattle when compared to passive surveillance. The targeted screening of fallen stock and emergency-slaughtered cattle considerably increased the number of detected cases in this 12-month period. Targeted-screening cases were on average 4 months younger than the clinical suspect cases. In conclusion, post-mortem testing of fallen stock and emergency-slaughtered cows >24 months for BSE is an important active surveillance element within a total surveillance system that principally is based on mandatory reporting of clinical suspect cases. Without ante-mortem screening tests to detect BSE-infected cattle during the incubation period, a combination of effectively functioning passive and active BSE surveillance strategies might be the only approach to assess the BSE situation reliably in a given country or region - and it is necessary to substantiate claims of freedom from the disease.
    NAL call no. SF601.P7
    Descriptors: cattle, bovine spongiform encephalopathy, screening, monitoring, diagnosis, identification, outbreaks, incidence, detection, efficacy, postmortem examinations, mortality, epidemiology, Switzerland.

80. Dominguez-Carmona, M. Epidemiologia de las enfermedades prionicas animales. [Epidemiology of the animal prion diseases] Anales de la Real Academia Nacional de Medicina (Madr.). 2001. 118(1): 233-245; discussion 245-258. ISSN: 0034-0634 In Spanish.
    Abstract: The authors review the epidemiology of spongiform encephalitis, discussing the possible mechanism of appearance of the epidemic bovine spongiform encephalitis epidemic, leaving aside to another publication the epidemiology of the human spongiform encephalitis and its pathogeny.
    Descriptors: review, epidemiology, BSE, spongiform encephalopathies.

81. Dormont, D. Les mecanismes de la mort neuronale. [Mechanisms of neuron death.] Biofutur. 2001, No. Hors Serie, 22-25; 7 ref. ISSN: 0294-3506. In French.
    NAL call no. TP248.13 B565
    Descriptors: BSE, apoptosis, bovine spongiform encephalopathy, central nervous system lesions, cytokines, free radicals, human diseases, neurons, pathogenesis, pathology, prion peptide 106-126, prion diseases.

82. Dove, A. US throws money at TSE research. Nature Medicine. 2001 Oct. 7(10): 1075 ISSN: 1078-8956
    Descriptors: US, transmissible spongiform encephalopathies, prion diseases, research funding initiatives.

83. Dumas, E., Cabre, O., Bornert, G. Mesures prises en France pour la prevention de la transmission de l'Encephalopathie Spongiforme Bovine. [French mandatory regulations to prevent transmission of Bovine Spongiform Encephalopathy.] Medecine et Armees. Juillet, 2001; 29 (4): 363-368. In French. ISSN: 0300-4937
    Descriptors: Bovine Spongiform Encephalopathy, BSE, food safety concern, mandatory regulations, prevention strategies, surveillance, use of animal proteins in animal feeds, screening tests.

84. Enders, M.; Frohlich, E.; Hassler, D.; Kretzschmar, H. BSE und die neue Variante der Creutzfeldt-Jakob-Krankheit. [BSE (bovine spongiform encephalopathy) and the new variant of Creutzfeldt-Jakob disease] Deutsche medizinische Wochenschrift 2001 Jan 26. 126(4): A55-A56 ISSN: 0012-0472
    NAL call no. 448.8 D48
    Descriptors: NvCreutzfeldt-Jakob Disease, BSE, relationship, human health risks.

85. Engvall, A.; Elvander, M. Riskvardering av lander avseende BSE. [Risk assessment of countries with regard to BSE.] Svensk Veterinartidning. 2001, 53: 8-9, 451-454; 4 ref. ISSN: 0346-2250 In Swedish.
    NAL call no. 41.9 SV23
    Descriptors: BSE, bovine spongiform encephalopathy, European Union, geographical distribution, human health risk assessment.

86. Enserink, M. Infectious diseases. Is the U.S. doing enough to prevent mad cow disease? Science. 2001 Jun 1. 292(5522): 1639-1641 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: BSE, scrapie, destruction of Vermont heard of sheep, prevention safeguards, border controls, prion diseases, chronic wasting disease in deer and elk, transmissible mink encephalopathy, testing measures, feed contamination, risk of bovine-based pharmaceuticals.

87. Enserink, M. Prion diseases. U.S. gets tough against chronic wasting disease. Science. 2001 Nov 2. 294(5544): 978-979 ISSN: 0036-8075
    NAL call no. 470 Sci2
    Descriptors: Deer, disease outbreaks, prion disease, wasting syndrome, animal husbandry, chronic disease, prevention and control.

88. Eskens, U. Bovine spongiform encephalopathy (BSE)/transmissible spongiform encephalopathy/mad cow disease. Environmental Science and Pollution Research International 2001. 8(2): 79-83 ISSN: 0944-1344
    Descriptors: BSE, issues, public health concerns, pollution concerns.

89. Estades, J.; Barbier, M.; Remy, E.; Aubert, F. (ed.); Sylvestre, J.P. TI: Le comite d'experts comme dispositif de production de confiance dans la gestion publique des risques: le cas de l'ESB. [The committee of experts as the mechanism for the production of confidence in the management of public risks: the case of BSE.] Confiance et rationalite, Dijon,-France, 5-6-mai 1999. 2001, 113-129; 31 ref. ISBN: 2-7380-0963-8. Publisher: Institut National de la Recherche Agronomique; Paris; France. In French.
    Descriptors: bovine spongiform encephalopathy, cattle diseases, food marketing, food safety, health, meat products, prion diseases, public opinion, public relations

90. FDA finds more evidence of BSE compliance problems. Journal of the American Veterinary Medical Assoc. 2001 Aug 15. 219(4): 427. ISSN: 0003-1488
    NAL call no. 41.8 Am3
    Descriptors: BSE, US feed industry, compliance in using animal based products, ruminant feeds.

91. Federici, C. La reazione del mercato alla vacca pazza. [Reaction of the Italian meat market to mad cow disease.] Rivista di Avicoltura. 2001, 70: 2, 8-10. ISSN: 0005-2213. In Italian.
    NAL call no. 47.8 R523
    Descriptors: beef consumption decline, BSE, bovine spongiform encephalopathy; pork and poultry meat increases, trends.

92. Ferguson-Smith, M.A. BSE and variant CJD. Assumption that BSE originated from scrapie in sheep led to misjudgment. BMJ Clinical Research ed. 2001 Jun 23. 322(7301): 1544-1545. ISSN: 0959-8138
    Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, scrapie, etiology, theories.

93. Foster, J. D.; Parnham, D.; Chong, A.; Goldmann, W.; Hunter, N. Clinical signs, histopathology and genetics of experimental transmission of BSE and natural scrapie to sheep and goats. The Veterinary Record 2001 Feb 10. 148(6): 165-171 ISSN: 0042-4900.
    Abstract: This paper compares the clinical signs, histopathology, detection of PrPSc protein and PrP genetics of the transmission of BSE to sheep and goats, with the effects of the transmission of natural scrapie from a brain homogenate from a single sheep. After intracerebral and oral inoculations there were similarities in the clinical signs due to the two sources of infection, but there were differences in pathology at the end stage of disease and in the genotypes of the sheep which succumbed to the challenges. The incubation period of BSE was associated with the sheep PrP codon 171 genotype, but the natural scrapie source, despite inducing disease only in known susceptible genotypes, showed no clear association with PrP genotype.
    NAL call no. 41.8 V641
    Descriptors: transmissibility, prion disease, scrapie, bovine spongiform encephalopathy, sheep, goats, histopathology, comparison study, PrP Sc, incubation period, behavior, genotype effects.

94. Foster, J. D.; Parnham, D.W.; Hunter, N.; Bruce, M. Distribution of the prion protein in sheep terminally affected with BSE following experimental oral transmission. Journal of General Virology. 2001 Oct. 82(Pt 10): 2319-2326 ISSN: 0022-1317
    Abstract: This study has examined the distribution of PrP(Sc) in sheep by immunocytochemistry of tissues recovered from terminally affected animals following their experimental infection by the oral route with BSE. Despite a wide range of incubation period lengths, affected sheep showed a similar distribution of high levels of PrP(Sc) throughout the central nervous system. PrP(Sc) was also found in the lymphoid system, including parts of the digestive tract, and some components of the peripheral nervous system. These abundant PrP(Sc) deposits in sheep in regions outside the central nervous system are in direct contrast with cattle infected with BSE, which show barely detectable levels of PrP(Sc) in peripheral tissues. A number of genetically susceptible, challenged animals appear to have survived.
    NAL call no. QR360.A1J6
    Descriptors: prion protein, PrP(Sc), immunocytochemistry, oral experimental infections, levels in lymphoid and nervous tissue.

95. Foster, J.; Goldmann, W.; Parnham, D.; Chong, A.; Hunter, N. Partial dissociation of PrPSc deposition and vacuolation in the brains of scrapie and BSE experimentally affected goats. J Gen Virol. Reading : Society for General Microbiology. Jan 2001. v. 82 (pt.1) p. 267-273. ISSN: 0022-1317
    Abstract: The diagnosis of transmissible spongiform encephalopathies (TSEs) depends on the detection of vacuolation in brain sections taken from affected individuals and/or the identification of the disease-associated isoform of the PrP (prion) protein (PrP(Sc)). During the course of an investigation, goats clinically affected following experimental infection with three different sources of TSE (SSBP/1, CH1641 and BSE) developed widespread vacuolar degeneration in the brain. With BSE, PrP(Sc) was clearly recognized in affected goat brain by immunocytochemistry (icc) and Western blotting, but in contrast the experimental scrapie sources SSBP/1 and CH1641 showed almost no or very little PrP(Sc) by icc. Western blot analysis of PrP(Sc) from BSE-affected and SSBP/1-affected goat brain showed that the protein was present in brain affected by both TSE sources, but could not be used to determine how much protein was present. It became clear that PrP(Sc) and vacuolation could be partially dissociated following challenge with two of the three TSE sources. Subtle differences in glycosylation patterns between BSE- and SSBP/1-associated PrP protein isoforms could also be recognized, although these experimentally generated results should not be regarded as a BSE/scrapie differential test. However, our study warns that the reliance on PrP(Sc) determination by icc alone as a means by which to diagnose TSE infection may generate false negative results.
    NAL call no. QR360.A1J6
    Descriptors: bovine spongiform encephalopathy, prion proteins, PrPSc, scrapie, goats, brain changes, diagnostic testing, reliability.

96. Fournier, J. G. Nonneuronal cellular prion protein. International Review of Cytology 2001. 208: 121-160 ISSN: 0074-7696
    Abstract: The normal cellular prion protein (PrP(c)) is a membrane sialoglycoprotein of unknown function having the unique property of adopting an abnormal tertiary conformation. The pathological conformer PrP(sc) would be the agent of transmissible spongiform encephalopathies or prion diseases. They include scrapie and bovine spongiform encephalopathy in animals and Creutzfeldt-Jakob disease in humans. The conversion of PrP(c) into PrP(sc) in the brain governs the clinical phenotype of the disease. However, the three-dimensional structure change of PrP(c) can also take place outside the central nervous system, in nonneuronal cells particularly of lymphoid tissue where the agent replicates. In natural infection, PrP(c) in nonneuronal cells of peripheral extracerebral organs may play a key role as the receptor required to enable the entry of the infectious agent into the host. In the present review we have undertaken a first evaluation of compelling data concerning the PrP(c)-expressing cells of nonneuronal origin present in cerebral and extracerebral tissues. The analysis of tissue, cellular, and subcellular localization of PrP(c) may help us better understand the biological function of PrP(c) and provide some information on physiopathological processes underlying prion diseases.
    NAL call no. 442.8 In82
    Descriptors: prion protein, nonneuronal cerebral and extracerebral organ receptors, physiopathological processes, prion diseases.

97. Fricker, J. BSE crisis--transmission through blood transfusions? Trends in Molecular Medicine. 2001 Jan. 7(1): 2-3 ISSN: 1471-4914
    Descriptors: BSE, risk factors, bovine-based pharmaceuticals, contamination of equipment.

98. Gabus, C.; Auxilien, S.; Pechoux, C.; Dormont, D.; Swietnicki, W.; Morillas, M.; Surewicz, W.; Nandi, P.; Darlix, J. The Prion Protein has DNA Strand Transfer Properties Similar to Retroviral Nucleocapsid Protein. Journal of Molecular Biology 2001 vol. 307, no. 4, pp. 1011-1021 ISSN: 0022-2836
    NAL call no. 442.8 J8224
    Descriptors: transmissible spongiform encephalopathies, PrP, nucleic acids, experimental infection of mice, PrP involvement in nucleic acid metabolism.

99. Gale, P. Developments on microbiological risk assessment for drinking water. J Appl Microbiol. Oxford, U.K. : Blackwell Science Ltd. Aug 2001. v. 91 (2) p. 191-205. ISSN: 1364-5072
    Abstract: This paper considers the development of microbiological risk assessment models for pathogenic agents in drinking water with particular reference to Cryptosporidium parvum, rotavirus and bovine spongiform encephalopathy (BSE). The available evidence suggests that there is potential for considerable variation in exposures to C. parvum oocysts through drinking water, during both outbreak and non-outbreak conditions. This spatial/temporal heterogeneity arises both from variation in oocyst densities in the raw water and fluctuations in the removal efficiencies of drinking water treatment. In terms of risk prediction, modeling the variation in doses ingested by individual drinking water consumers is not important if the dose-response curve is linear and the oocysts act independently during infection. Indeed, the total pathogen loading on the population as represented by the arithmetic mean exposure is sufficient for risk prediction for C. parvum, BSE and other agents of low infectivity, providing the infecting particles (i.e. oocysts or BSE prions) are known to act independently. However, for more highly infectious agents, such as rotavirus, ignoring the variation and just using the arithmetic mean exposure may over-estimate the risk by a factor of about threefold. If it were to be shown that pathogens co-operate with each other during initiation of infection, such that the dose-response relationship is non-linear, then modelling the variation in doses ingested by individual consumers would be very important. Possible mechanisms for co-operation of pathogens during infection are considered. Simulations show that acquired protective immunity for C. parvum reduces the risk of infection during outbreak conditions by over 10-fold. Variation in virulence between strains of C. parvum is a further source of uncertainty.
    NAL call no. QR1.J687
    Descriptors: pathogens, water-microbiology, risks of BSE contamination.

100. Gale, P.; Stanfield, G. Towards a quantitative risk assessment for BSE in sewage sludge. Journal of Applied Microbiology. September, 2001; 91 (3): 563-569. ISSN: 1364-5072
     NAL call no. QR1 J687
     Descriptors: Source-Pathway-Receptor approach, BSE, contaminated waste, arithmetic mean concentration of BSE agent, slaughter house effluent, horizontal transmission risk.

101. Garcia, B.M. Encefalopatia espongiforme bovina. [Bovine spongiform encephalopathy.] Alimentaria. 2001, No. Extr., 24-62; 32 ref. ISSN: 0300-5755. In Spanish.
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, Creutzfeldt-Jakob disease, human diseases, nervous system diseases, prion diseases, reviews.

102. Gatnau R.; Polo J.; Robert, E.; Brufau J. Plasma protein antimicrobial substitution at negligible risk. Feed manufacturing in the Mediterranean region. Improving safety: from feed to food. Proceedings of the III Conference of Feed Manufacturers of the Mediterranean, organized by ASFAC, with the collaboration of IRTA, CESFAC, CIHEAM, Patronat Catala Pro Europa and Fira de Reus, and sponsored by Port de Tarragona, Reus (Spain), 22-24 March 2000. Cahiers Options Mediterraneennes. 2001, 54: 141-150; 45 ref. ISSN: 1022-1379. In French with an English summary.
     Descriptors: blood derived proteins, feed ingredients, disease risks, BSE, dioxin, biological safety, correlating blood derived products and health benefits, risk assessment.

103. Gavier Widen, D.; Wells, G.A.; Simmons, M. M.; Wilesmith, J.W.; Ryan, J. Histological observations on the brains of symptomless 7-year-old cattle. Journal of Comparative Pathology. 2001 Jan. 124(1): 52-59 ISSN: 0021-9975
     Abstract: The histological changes in the brains of 506 clinically normal 7-year-old cattle, which were part of a cohort study on maternal transmission of bovine spongiform encephalopathy, are described. Vacuolation of the white matter, of unknown aetiology, located particularly in the substantia nigra, was a frequent finding. Vacuolated neurons were commonly observed in the red nucleus (64.3% of the animals) and in the habenular nucleus (50.1%). Spheroids were found in 10.8% of the brains, most frequently in the vestibular nuclei. Cellular inflammatory infiltrates in association with blood vessels occurred in 30% of the animals at various locations in the brain; their aetiology remains uncertain, but they may have reflected subclinical or latent infections. Mineralization of the wall of blood vessels, with proliferation of the intima, was observed frequently in vessels of the internal capsule and was probably associated with ageing. The description of histological findings in the brain of symptomless adult cattle in the present study provides a useful background for diagnostic bovine neuropathology.
     NAL call no. 41.8 J82
     Descriptors: histological changes, normal 7 year old cattle, maternal BSE transmission, vacuolated neurons, substantia nigra.

104. Gee, R.W. Communication of risk. Australian Veterinary Journal. 2001 Feb. 79(2): 136. ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: zoonotic diseases, TSE, media and governmental communications, BSE, UK, Australia.

105. Germany. Gesellschaft fuer Tierzuchtwissenschaft Position statement of the Gesellschaft fuer Tierzuchtwissenschaft (GfT) (Society for Animal Husbandry Science) and the Deutsche Gesellschaft fuer Zuechtungskunde (DGfZ) (German Society for Animal husbandry) regarding the BSE crisis (Bovine Spongiform Encephalitis). Zuechtungskunde. Marz-April, 2001; 73 (2): 81-84. ISSN: 0044-5401. In German.
     NAL call no. 49 Z8
     Descriptors: BSE, Germany societies position paper.

106. Giles, J. Mad cow disease comes to Japan. Nature. 2001 Sep 20. 413(6853): 240 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: cattle, BSE, confirmed case, imported feed, contaminated feed, UK, Japan.

107. Glatzel, Markus; Aguzzi, Adriano. The shifting biology of prions. Brain Research Reviews. October, 2001; 36 (2-3): 241-248. ISSN: 0165-0173.
     Descriptors: BSE, transmissible spongiform encephalopathies, prion and prion diseases.

108. Goldwater, P.N. Bovine spongiform encephalopathy and variant Creutzfeldt-Jakob disease: implications for Australia. Medical Journal of Australia, 2001 Aug 6. 175(3): 154-158 ISSN: 0025-729X
     Abstract: The bovine spongiform encephalopathy (BSE) epizootic developed in the United Kingdom in the mid-1980s. Feeding practices in the cattle industry amplified the causative prion, and meat contaminated with BSE entered the market. Human consumption of prion-contaminated meat led to the new zoonosis--variant Creutzfeldt-Jakob disease (vCJD). The UK BSE Inquiry published its report in October 2000; while praising policy decisions, it also documented failures in the execution of these policies, specifically delays and lack of rigour. Australia is in an excellent position to maintain its BSE- and scrapie-free status, but widespread active surveillance of neural and non-neural tissue from all species of farmed quadrupeds is needed.
     Descriptors: disease free status, BSE, scrapie, Australia.

109. Golsteyn, R. M. When being right is not enough. Trends in Cell Biology 2001 Feb. 11(2): 59 ISSN: 0962-8924
     NAL call no. QH573.T73
     Descriptors: Bovine spongiform encephalopathy, communication, transmission, politics, cattle.

110. Grant, H. BSE and variant CJD. Humans can live with BSE so long as they do not eat brains. BMJ-Clinical Research ed. 2001 Jun 23. 322(7301): 1545. ISSN: 0959-8138
     Descriptors: bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, scrapie, etiology, theories, diet, human health risks.

111. Gravenor, M. B.; Cox, D. R.; Hoinville, L. J.; Hoek, A.; McLean, A.R. The flock-to-flock force of infection for scrapie in Britain. Proceedings of the Royal Society of London. Series B. Biological Sciences. 2001 Mar 22. 268(1467): 587-592 ISSN: 0962-8452
     Abstract: A postal survey of British sheep farmers provided information on the proportion of farms that experienced their first case of scrapie in each year between 1962 and 1998. We found no evidence of a large increase in the proportion of scrapie-affected farms prior to, during or following the epidemic of BSE in British cattle. After correcting for between-farm heterogeneity in the probability of acquiring scrapie, we estimated the yearly between-flock force of infection since 1962. The current force of infection is estimated at approximately 0.0045 per farm per year and combined with a simple model of scrapie spread provides an estimate of the average duration of a scrapie outbreak on an individual farm. Considering all farms, the average outbreak lasts for five years, but if only those farms that have cases in animals born on the farm are considered, it lasts 15 years. We use these parameter estimates to compare the proportion of farms with scrapie in time periods of different lengths. In the survey, 2.7% of farms had a case in 1998. The 5.3% of farms reporting having a case between 1993 and 1997 is consistent with the hypothesis that the scrapie force of infection remained constant over this period.
     NAL call no. 501 L84B
     Descriptors; Disease outbreaks, scrapie transmission, epidemiology, prevention and control, sheep, diseases, cattle, bovine spongiform encephalopathy, Great Britain, statistical models, questionnaires.

112. Griffiths, P.D. Variant CJD epidemiology: joining up the dots. Reviews in Medical Virology 2001 vol. 11, no. 4, pp. 203-204 ISSN: 1052-9276.
     Descriptors: BSE, disease transmission, NvCreutzfeldt-Jakob-disease, incidence of disease, mathematical model of human exposure, disease projection.

113. Gross, John E.; Miller, Michael W. Chronic wasting disease in mule deer: Disease dynamics and control. Journal of Wildlife Management. Bethesda, MD: The Wildlife Society. April 2001 v. 65 (2) p. 205-215. ISSN: 0022-541X
     Abstract: The authors developed a mechanistic model to simulate the dynamics of CWD in mule deer populations. Estimated parameters were projected for age-specific disease dynamics, changes in population size and effects of control strategies. The parameters were estimated from observations of infections and uninfected deer in Colorado. They found that the culling rate (20% of infected populations) effectively eliminated the disease at low disease levels. When disease rates were high, the likelihood of disease control diminished rapidly. To eliminate CWD from wild populations may take decades.
     NAL call no. 41.9 W64B
     Descriptors: chronic wasting disease, (CWD) transmissible spongiform encephalopathies, Odocoileus hemionus, mule deer, PrPres, prion protein, epidemiology, game-farmed wapiti, disease control measure, wildlife populations, wildlife management, public health threats, mathematical model, Monte Carlo techniques

114. Gunn, M. Observations on disposal of BSE-infected carcasses. Irish Veterinary Journal. 2001, 54: 4, 192-193; 4 ref. ISSN: 0368-0762
     NAL call no. 41.8 IR4
     Descriptors: BSE, bovine spongiform encephalopathy, brain, carcass disposal, sources of infection, human risk assessment.

115. Guo, Zhi Ru; Jin, NingYi; Guo, Z.R.; Jin, N.Y. Advances in research on bovine spongiform encephalopathy. Chinese Journal of Veterinary Science. 2001, 21: 3, 307-311; 49 ref. ISSN: 1005-4545. In Chinese.
     NAL call no. SF604.J68
     Descriptors: Bovine spongiform encephalopathy, research, reviews.

116. Half a response. Vet Rec. London : The British Veterinary Association. Feb 17, 2001. v. 148 (7) p. 189. ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: bovine spongiform encephalopathy, guidelines, UK.

117. Hamir, A.N.; Cutlip, R.C.; Miller, J.M.; Williams, E.S.; Stack, M.J.; Miller, M.W.; O'Rourke, K.I.; Chaplin, M.J. Preliminary findings on the experimental transmission of chronic wasting disease agent of mule deer to cattle. J Vet Diagn Invest. Lawrence, Kan. : AAVLD. Jan 2001. v. 13 (1) p. 91-96. ISSN: 1040-6387
     NAL call no. SF774.J68
     Descriptors: cattle, mule deer, chronic wasting disease, Odocoileus hemionus, spongiform encephalopathy, disease transmission, experimental infections, disease course, brain lesions, diagnostic techniques, prion proteins.

118. Hammer, G.F. Massnahmen-Bundel fur mehr Sicherheit: die tierseuchen- und fleischhygienerechtliche Vorschriften zur BSE-Bekampfung. [Combined measures for more security: the regulations relating to animal epidemics and meat hygiene for BSE control.] Fleischwirtschaft. 2001, 81: 4, 54-60; 25 ref. ISSN: 0015-363X. In German.
     NAL call no. 280.38 F62
     Descriptors: animal health, BSE, bovine spongiform encephalopathy, cattle, meat product safety measures, prion diseases, regulations, Germany.

119. Haunhorst E. Lebensmitteluberwachung: Umsetzung der BSE-Regelungen Massnahmen Massnahmen der Lander sollen Schutz der Verbraucher gewahrleisten. [Implementation of BSE regulations to protect consumers.] Fleischwirtschaft. 2001, 81: 6, 20-22. ISSN: 0015-363X. In German.
     NAL call no. 280.38 F62
     Descriptors: BSE, bovine spongiform encephalopathy; legislation implementation, German provinces.

120. Healy, B. vCJD: broad U.S. response required. Science. 2001 Mar 9. 291(5510): 1859 ISSN: 0036-8075
     NAL call no. 470 Sci2
     Descriptors: Creutzfeldt-Jakob Syndrome, diagnosis, prevention and control, cattle, lymphoid tissue, population surveillance, United States.

121. Heeschen, W.H. Bovine Spongiforme Enzephalopathie (BSE). "Milch ist als sicher anzusehen" -- derzeitiger Stand wissenschaftlicher Erkenntnisse zu dieser Aussage. [Bovine spongiform encephalopathy (BSE). "Milk is safe" -- current state of scientific results regarding this quote.] DMZ, Lebensmittelindustrie und Milchwirtschaft. 2001, 122: 2, 60-67; 17 ref. ISSN: 0938-9369. In German.
     NAL call no. HD9275 G3D59
     Descriptors: milk, food safety, food contamination, human health risk assessment, BSE, bovine spongiform encephalopathy; cattle prion diseases.

122. Heppner, Frank L.; Arrighi, Isabelle; Kalinke Ulrich; Aguzzi, Adriano. Immunity against prions? Trends in Molecular Medicine. November, 2001; 7 (11): 477-479. ISSN: 1471-4914
     Descriptors: prion protein, antibodies, scrapie-infected cells in vitro, feasibility of immunotherapeutical intervention, question effectiveness of vaccines and post-exposure strategies based on antibodies scrapie, BSE, Creutzfeldt-Jakob disease

123. Heppner, F. L.; Musahl, C.; Arrighi, I.; Klein, M. A.; Rulicke, T.; Oesch, B.; Zinkernagel, R. M.; Kalinke, U.; Aguzzi, A. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies. Science. 2001 Oct 5. 294(5540): 178-182 ISSN: 0036-8075
     Abstract: Variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy are initiated by extracerebral exposure to prions. Although prion transmission from extracerebral sites to the brain represents a potential target for prophylaxis, attempts at vaccination have been limited by the poor immunogenicity of prion proteins. To circumvent this, we expressed an anti-prion protein (anti-PrP) mu chain in Prnp(o/o) mice. Transgenic mice developed sustained anti-PrP titers, which were not suppressed by introduction of Prnp+ alleles. Transgene expression prevented pathogenesis of prions introduced by intraperitoneal injection in the spleen and brain. Expression of endogenous PrP (PrP(C)) in the spleen and brain was unaffected, suggesting that immunity was responsible for protection. This indicates the feasibility of immunological inhibition of prion disease in vivo.
     NAL call no. 470 Sci2
     Descriptors: prion diseases, anti-prion protein, mouse model, introperitoneal injection, spleen, brain, immunity response, possibilities for effective vaccine.

124. Hill, A.F.; Collinge, J.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Strain variations and species barriers. Prions: a challenge for science, medicine and public health-system. 2001, 48-257; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, Creutzfeldt Jakob disease, prion diseases, scrapie, strain differences, species barriers.

125. Hof, G. Verwarring rond boviene spongiforme encefalopathie (BSE) en het risico op de nieuwe variant van de ziekte van Creutzfeldt-Jakob. [Confusion surrounding bovine spongiform encephalopathy (BSE) and the risk of new variant Creutzfeldt-Jakob disease] Nederlands Tijdschrift voor Geneeskunde 2001 Mar 10. 145(10): 501-502. ISSN: 0028-2162. In Dutch.
     Descriptors: BSE, transmissible spongiform encephalopathies, NvCreutzfeldt-Jakob disease, human health risks, exposure to contaminated bovine-based products.

126. Hossain, H.; Chakraborty, T. Prion-Krankheiten. [Prion diseases] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Jan. 36(1): 15-24 ISSN: 0939-2661. In German.
     Descriptors: prions, prion diseases, BSE, CJD, human health risks.

127. Huillard-d'Aignaux, J. N.; Cousens, S.N.; Smith, P.G. Predictability of the UK variant Creutzfeldt-Jakob disease epidemic. Science. 2001 Nov 23. 294(5547): 1729-1731 ISSN: 0036-8075
     Abstract: Back-calculation analysis of the variant Creutzfeldt-Jakob disease epidemic in the United Kingdom is used to estimate the number of infected individuals and future disease incidence. The model assumes a hazard of infection proportional to the incidence of bovine spongiform encephalopathy in the United Kingdom and accounts for precautionary control measures and very wide ranges of incubation periods. The model indicates that current case data are compatible with numbers of infections ranging from a few hundred to several millions. In the latter case, the model suggests that the mean incubation period must be well beyond the human life-span, resulting in disease epidemics of at most several thousand cases.
     NAL call no. 470 Sci2
     Descriptors: BSE, back-circulation analysis model, epidemiology, human health risks, disease incidence projection

128. Investigation of vCJD cluster points to butchering practices. Clinical Infectious Diseases 2001 Jun 15. 32(12): ii. ISSN: 1058-4838
     NAL call no. RC111 R4
     Descriptors: cattle slaughter processes, BSE, NvCreutzfeldt-Jakob Disease, incidence, disease transmission risks.

129. Jackson, G.S.; Collinge, J. The molecular pathology of CJD: old and new variants. Mol Pathol. 2001 Dec. 54(6): 393-399 ISSN: 1366-8714
     Abstract: The study of prion disease has become an area of intense interest since experimental evidence emerged for the transmission of phenotypic variation without the involvement of a nucleic acid component. Additional impetus has come from the widespread concern that exposure to bovine spongiform encephalopathy contaminated material poses a distinct and, conceivably, a severe threat to public health in the UK and other countries. The occurrence of new variant Creutzfeldt-Jakob disease has dramatically highlighted the need for a precise understanding of the molecular basis of prion propagation. The molecular basis of prion strain diversity, previously a major challenge to the "protein only" model, can now be reconciled with propagation of infectious protein topologies. The conformational change known to be central to prion propagation, from a predominantly alpha-helical fold to one predominantly comprising beta-structure, can now be reproduced in vitro, and the ability of beta-PrP to form fibrillar aggregates provides a plausible molecular mechanism for prion propagation. Concomitantly, advances in the fundamental biology of prion disease have done much to reinforce the protein only hypothesis of prion replication.
     Descriptors: BSE exposure, public health concerns, NvCreutzfeldt-Jakob Disease, prion proteins, conversion to bets helical structure, in vitro.

130. Jackson, G.S.; Beck, J.A.; Navarrete, C.; Brown, J.; Sutton, P.M.; Contreras, M.; Collinge, J. Pathogenesis: HLA-DQ7 antigen and resistance to variant CJD Nature 2001 vol. 414, no. 6861, pp. 269-270 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: NvCreutzfeldt-Jakob disease, bovine spongiform encephalopathy (BSE)-like prion strain, human leukocyte antigen, differential diagnosis, host susceptibility.

131. Japan's beef scandal. Nature. 2001 Sep 27. 413(6854): 333 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: BSE, bovine spongiform encephalopathy, NvCreutzfeldt-Jakob Disease, biased government communications, Japan, confirmed case, cattle, feed contamination concerns, UK, meat and bone meal imports.

132. Jeffrey M.; Martin, S.; Gonzalez, L.; Ryder, S.J.; Bellworthy, S.J; Jackman, R. Differential diagnosis of infections with the bovine spongiform encephalopathy (BSE) and scrapie agents in sheep. Journal Comparative Pathology. November, 2001; 125 (4): 271-284. ISSN: 0021-9975
     NAL call no. 41.8 J82
     Descriptors: scrapie, bovine spongiform encephalopathy, BSE, NvCreutzfeldt-Jakob disease, transmissible spongiform encephalopathies, prion diseases, sheep, immunohistochemical methods, brain, lymphoreticular system, experimental and natural infections, strain-dependent processing, strain identification.

133. Jeffrey, M.; Ryder, S.; Martin, S.; Hawkins, S.A.C.; Terry, L.; Berthelin-Baker, C.; Bellworthy, S.J. Oral inoculation of sheep with the agent of bovine spongiform encephalopathy (BSE). 1. Onset and distribution of disease-specific PrP accumulation in brain and viscera. J Comp Pathol. London : W.B. Saunders Company Ltd. May 2001. v. 124 (4) p. 280-289. ISSN: 0021-9975
     Abstract: Sixty-three Romney sheep aged 6 months, consisting of three groups (PrP(ARQ/ARQ), PrP(ARQ/ARR), and PrP(ARR/ARR)genotypes) of 21 animals, were infected orally with brain tissue from BSE-infected cattle. Sub-groups of the 21 PrP(ARQ/ARQ) animals were killed, together with uninfected controls 4, 10, 16, 22 or 24-28 (after the development of full clinical disease) months post-inoculation (mpi). One sheep from each of the two groups of four killed at 4 or 10 mpi were shown by immunohistochemical examination to possess disease-specific PrP accumulations in single lymph nodes. At 16 mpi, such accumulations were detected in two of four infected sheep in some viscera and in the spinal cord and brain. At 22 mpi, three of five infected sheep had widespread disease-specific PrP accumulations in all tissues examined, but the remaining two animals gave positive results only in the central nervous system. Clinical disease appeared at 20-28 mpi. Three sheep killed with advanced clinical signs showed widespread PrP accumulation in brain, spinal cord and peripheral tissues. These results confirmed that PrP(ARQ/ARQ) Romney sheep are susceptible to experimental infection with the BSE agent. The different sites at which initial PrP accumulations were detected suggested that the point of entry of infection varied. Once established, however, infection appeared to spread rapidly throughout the lymphoreticular system. The results suggested that in some BSE-infected sheep neuroinvasion occurred in the absence of detectable PrP accumulations in the viscera or peripheral nervous system. In contrast to cattle with BSE, however, most sheep showed disease-specific PrP accumulations in the lymphoreticular system. In this respect, BSE-infected resembled scrapie-infected sheep; it is possible, however, that future research will reveal differences in respect of targeting of cell types within the lymphoreticular and peripheral nervous systems. The PrP(ARQ/ARR)and PrP(ARR/ARR)sheep were also killed in sub-groups at intervals after inoculation. Up to 24 mpi, however, none of these animals showed disease-specific PrP accumulations. Further results will be reported later.
     NAL call no. 41.8 J82
     Descriptors: sheep, bovine spongiform encephalopathy, genotypes, prion proteins, brain experimental infection, spinal cord, animal tissues, clinical aspects, pathogenesis, peripheral nerves.

134. Kaaden, O.R.; Rabenau, H.F. (ed.); Cinatl, J. (ed.); Doerr, H.W. Animal transmissible spongiform encephalopathy: clinical and diagnostic aspects. Prions: a challenge for science, medicine and public health-system. 2001, 145-150; 48 ref. Published by: S. Karger AG; Basel; Switzerland. ISBN: 3-8055-7124-0
     NAL call no. QR1 C66 v. 7
     Descriptors: BSE, bovine spongiform encephalopathy, clinical aspects, diagnosis, pathogenesis, prion diseases, scrapie, animals.

135. Kahrs, R.F. Viral diseases of cattle. 2001, viii + 324 pp. Iowa State University Press; Ames; USA ISBN: 0-8138-2591-1
     NAL call no. SF961 K26 2001
     Descriptors: Aujeszky's disease, bovine leukosis, BSE, bovine spongiform encephalopathy, clinical aspects, disease diagnosis, disinfectants, diagnostic-techniques, FMD, foot and mouth disease, rabies, international trade, malignant catarrhal fever, rhinotracheitis, Rift Valley fever, rinderpest, vaccines, vulvovaginitis, various viral diseases, herpes, warts, poxvirus, etc.

136. Kamphues, J.; Zentek, J.; Oberthur, R.C.; Flachowsky, G.; Coenen, M. Futtermittel tierischer Herkunft als mogliche Verbreitungsursache fur die bovine spongiforme Enzephalopathie (BSE) in Deutschland: 1. Mitteilung: Vergleichende Risikobewertung der Einzelfuttermittel tierischer Herkunft. [Animal-derived feeds as possible vectors for bovine spongiform encephalopathy (BSE) in Germany. 1. Comparative risk assessment for a single animal food of animal origin] Deutsche Tierarztliche Wochenschrift 2001 Jul. 108(7): 283-290. ISSN: 0341-6593. In German.
     Abstract: The occurrence of BSE cases in Germany after the ban of meat and bone meal for ruminant feed in 1994 requires a detailed investigation of animal derived feedstuffs regarding their specific risks as vectors for the disease. Accepting the theory that BSE is a prion transmitted disease, the theoretical infectious potential was calculated for animal derived feedstuffs. This calculation was based on the assumption, that risk material (brain, spinal cord) of one clinically diseased cattle was rendered in the process as established in Germany (133 degrees C, 3 bar, 20 min) or, alternatively, that one diseased animal was slaughtered resulting in normal processing of the by-products for human food production. From this risk assessment it became obvious that meat and bone meal was one, but probably not the most important source for the spreading of BSE. Taking into account the high sensitivity of calves it can be speculated that certain products, e.g. from bone processing (bone meal) and fat melting (mixed animal fats), commonly used for the formulation of milk replacers, might have been more important as pathways. As it can't be excluded retrospectively that infected meat and bone meal was imported from the UK, this non-calculable influence may have been related to the significance of the other products. The calculation model underlines that efficient removal of specified risk material (brain, spinal cord) and adequate processing (133 degrees C, 3 bar, 20 min) or alternatively other equivalent treatments of fats are prerequisites for minimizing the risk of feed borne transmission of BSE by animal derived feedstuffs. The epidemiological consequences are part of a subsequent paper.
     NAL call no. 41.8 D482
     Descriptors: contaminated feeds, meat and bone meal, feed processing, cattle, risk assessment.

137. Kingombe, Cesar Isigidi Bin; Luthi, Elisabeth; Schlosser, Heidi; Howald, Denise; Kuhn, Monika; Jemmi, Thomas. A PCR-based test for species-specific determination of heat treatment conditions of animal meals as an effective prophylactic method for bovine spongiform encephalopathy. Meat Science. January, 2001; 57 (1): 35-41. ISSN: 0309-1740
     NAL call no. TX373 M4
     Descriptors: animal waste sterilization, animal based feeds, ELISA and PCR assays, pork tissue, bovine tissue, screening method.

138. Kleinhanss W.; Haxsen G.; Uhlmann F.; Kamphues J. (ed.); Flachowsky G. Okonomische Bewertung eines Verbots der Tiermehlherstellung und verfutterung. [Economic assessment of a ban on meat and bone meal production and feeding.] Animal nutrition - resources and future developments. Workshop on Sustainable Animal Production, 15-16 June 2000, EXPO 2000, Hannover, Germany. Landbauforschung-Volkenrode,-Sonderheft. 2001, No. 223, 159-166; 1 ref. ISSN: 0376-0723 ISBN: 3-933140-47-1. In German with an English summary.
     Descriptors: economic impact, alternative rendering system, ban on meat and bone meals, waste disposal, carcass disposal, BSE.

139. Klimuszko, D. Priony -- czynnik etiologiczny gabczastych encefalopatii. [Prions in the aetiology of spongiform encephalopathies.] Zycie Weterynaryjne. 2001, 76: 3, 128-130; 14 ref. ISSN: 0137-6810. In Polish.
     NAL call no. SF604.Z9
     Descriptors: BSE, etiology, bovine spongiform encephalopathy, prion diseases, pathogenesis, public health risks, scrapie.

140. Kolb, E. Neuere Erkenntnisse uber die Eigenschaften des Erregers der BSE und der Traberkrankheit sowie uber die Infektionswege (Ubersichtsreferat). [Recent studies on the properties of BSE and scrapie infectious agents and mechanisms of infection.] Tierarztliche Umschau. 2001, 56: 4, 175-183; 37 ref. ISSN: 0049-3864. In German with an English summary.
     NAL call no. 41.8 T445
     Descriptors: BSE, bovine spongiform encephalopathy, prion disease, scrapie, sheep, cattle, disease process.

141. Koo, Hye Cheong; Park, Yong Ho; Lee, Byeong Chun; Chae, Chan Hee; O' Rourke K.I.; Baszler, T.V.; Koo, H.C.; Park Y.H.; Lee, B.C.; Chae, C.H. Immunohistochemical detection of prion protein (PrP-Sc) and epidemiological study of BSE in Korea. Journal of Veterinary Science. 2001, 2: 1, 25-31; 43 ref. ISSN: 1229-845X
     NAL call no. SF604.J68
     Descriptors: BSE, etiology, prion protein, cattle survey, Korea, immunohistochemistry and western immunoblotting assays, epidemiology.

142. Koppinen, J. The French reflect on BSE. Australian Veterinary Journal 2001 Jul. 79(7): 452 ISSN: 0005-0423
     NAL call no. 41.8 Au72
     Descriptors: BSE, the French situation.

143. Kramer, G. N.; Pauwels, J.; Le Guern, L.; Schimmel, H.; Trapmann, S. Recent production of candidate reference materials at IRMM. Fresenius' Journal of Analytical Chemistry. 2001 Jun. 370(2-3): 142-146 ISSN: 0937-0633
     Abstract: In the execution of its mission to promote a common European measurement system in support of EU policies, IRMM's Reference Materials Unit is currently involved in preparation of proficiency-testing samples and candidate reference materials. Recent work related to bovine spongiform encephalopathy in cows, genetically modified organisms, and a variety of environmental materials is described.
     NAL call no. QD71.F7
     Descriptors: EU policies, BSE, measurement systems, reference materials.

144. Krammer, R. BSE crisis sinks German public biotech programme. Nature. 2001 Feb 1. 409(6820): 549 ISSN: 0028-0836
     NAL call no. 472 N21
     Descriptors: bovine spongiform encephalopathy, impacts on research, biotechnology research, Germany.

145. Krcmar, P.; Rencova, E. Identification of bovine-specific DNA in feedstuffs. Journal of food protection 2001 Jan. 64(1): 117-119 ISSN: 0362-028X
     Abstract: Considering the menace of transmission of bovine spongiform encephalopathy, feed components intended for cattle nutrition must be checked for the presence of bovine-derived materials. We have been using a method based on polymerase chain reaction for the identification of bovine-specific mitochondrial DNA sequences for this purpose. The specificity of the primers for polymerase chain reaction has been tested using samples of DNA of other vertebrate species, which may also be present in rendering plant products. The method allows the detection in concentrate mixtures of 0.125% of bovine-derived material. Bovine DNA at concentrations corresponding to less than 0.5% of bovine-derived material was detected in 3 of the 30 samples of concentrate mixtures collected from distributors' stores all over the Czech Republic. All 44 samples of fish meal collected from the same sources were free of bovine-derived material.
     NAL call no. 44.8 J824
     Descriptors: BSE, bovine derived feed testing, feed contamination, mitochondrial DNA testing, polymerase chain reaction method, detection method.

146. Kuzma, C.D. BSE: could it happen here? Experts say probably not. Journal of the American Veterinary Medical Association. 2001 Mar 1. 218(5): 646-647, 650 ISSN: 0003-1488
     NAL call no. 41.8 Am3
     Descriptors: bovine spongiform encephalopathy, cattle, US.

147. Laffling, A.J.; Baird, A.; Birkett, C.R.; John, H.A. A monoclonal antibody that enables specific immunohistological detection of prion protein in bovine spongiform encephalopathy cases. Neuroscience Letters. 2001 Mar 9. 300(2): 99-102 ISSN: 0304-3940
     Abstract: The specificity of a monoclonal antibody (mAB) raised against recombinant bovine prion protein (PrP) for the immunohistological detection of PrP accumulation in the medulla oblongata of bovine spongiform encephalopathy (BSE) and ovine scrapie cases was investigated. mAB KG9 showed a diffuse low intensity reaction with the cytoplasm of neurones in normal cattle and sheep sections. In BSE sections the mAB detected widespread granular deposits of PrP associated with neurones and the neuropil. Although scrapie sections showed similar levels of granular deposits with another antibody to PrP these were not detected by KG9 which did however detect diffuse staining in neuronal cytoplasm. Possible explanations for the specificity of binding of KG9 are discussed.
     NAL call no. QP351 N3
     Descriptors: BSE, diagnosis, cattle, medulla oblongata, ovine scrapie, mAB KG9 antibody, PrP, specificity of KG 9 binding.

148. Lahiff, S.; Glennon, M.; O' Brien, L.; Lyng, J.; Smith, T.; Maher, M.; Shilton, N. Species-specific PCR for the identification of ovine, porcine and chicken species in meat and bone meal (MBM). Molecular and Cellular Probes. February, 2001; 15 (1): 27-35. ISSN: 0890-8508
     NAL call no. RB43.7.M63
     Descriptors: silica-guanidiumthiocyanate DNA isolation procedure, commercial DNA extraction kit, identification of source materials, animal feed formulations, tissue identification, sheep, swine, poultry, transmissible spongiform encephalopathies.

149. Langenfeld, T.W.; Menges, T.; Hempelmann, G. Bovine spongiforme Enzephalopathie (BSE). [Bovine spongiform encephalopathy (BSE)] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Feb. 36(2): 77-78 ISSN: 0939-2661. In German
     Descriptors: BSE, aspects of the disease, cattle.

150. Lasky, Tamar; Etzel, Ruth; Angulo, Fred; Ward, Hester; Powell, Mark; Rubin, Carol Food safety: Challenges to epidemiology. American Journal of Epidemiology. June 1, 2001; 153 (11 Supplement): S13. ISSN: 0002-9262. Joint Meeting of the Society for Epidemiologic Research, American College of Epidemiology, Epidemiology Section of the American Public Health Association, and the Canadian Society for Epidemiology and Biostatistics, Toronto, Canada, June 13-16, 2001
     NAL call no. 449.8 Am3
     Descriptors: food safety, emerging diseases, public health risks, BSE.

151. Lasmezas, C.I.; Fournier, J.G.; Nouvel, V.; Boe, H.; Marce, D.; Lamoury, F.; Kopp, N.; Hauw, J.J.; Ironside, J.; Bruce, M. Adaptation of the bovine spongiform encephalopathy agent to primates and comparison with Creutzfeldt-Jakob disease: implications for human health. Proc Natl Acad Sci, USA. Washington, D.C. : National Academy of Sciences, Mar 27, 2001. v. 98 (7) p. 4142-4147. ISSN: 0027-8424
     Abstract: There is substantial scientific evidence to support the notion that bovine spongiform encephalopathy (BSE) has contaminated human beings, causing variant Creutzfeldt-Jakob disease (vCJD). This disease has raised concerns about the possibility of an iatrogenic secondary transmission to humans, because the biological properties of the primate-adapted BSE agent are unknown. We show that (i) BSE can be transmitted from primate to primate by intravenous route in 25 months, and (ii) an iatrogenic transmission of vCJD to humans could be readily recognized pathologically, whether it occurs by the central or peripheral route. Strain typing in mice demonstrates that the BSE agent adapts to macaques in the same way as it does to humans and confirms that the BSE agent is responsible for vCJD not only in the United Kingdom but also in France. The agent responsible for French iatrogenic growth hormone-linked CJD taken as a control is very different from vCJD but is similar to that found in one case of sporadic CJD and one sheep scrapie isolate. These data will be key in identifying the origin of human cases of prion disease, including accidental vCJD transmission, and could provide bases for vCJD risk assessment.
     NAL call no. 500 N21P
     Descriptors: bovine spongiform encephalopathy, prions, intravenous injection, disease transmission, Macaca fascicularis, symptoms, mice, Creutzfeldt-Jakob Disease, cerebellum, cerebral cortex, histopathology, immunohistochemistry, scrapie, strains.

152. Lawler, S. Farmer's research goes against the grain. Trends in Cell Biology 2001 Mar. 11(3): 110. ISSN: 0962-8924
     NAL call no. QH573.T73
     Descriptors: Bovine spongiform encephalopathy, chemically induced, insecticides, organothiophosphate poisoning, cattle, copper and manganese chemistry, prions, intravenous, drug effects.

153. Lledo, Pierre-Marie. Histoire de la vache folle. [‘The history of mad cow.”]. Science, histoire et societe. Paris : Presses Universitaires de France, c2001. xvi, 158, [1] p., ill. In French. ISBN: 2130515738
     NAL call no. SF967.S63 L58 2000
     Descriptors: bovine spongiform encephalopathy, prion diseases, animals prions disease history.

154. Lloyd, S.E.; Onwuazor, O.N.; Beck, J.A.; Mallinson, G.; Farrall, M.; Targonski, P.; Collinge, J.; Fisher, E.M.C. Identification of multiple quantitative trait loci linked to prion disease incubation period in mice Proceedings of the National Academy of Sciences, USA 2001 vol. 98, no. 11, pp. 6279-6283 ISSN: 0027-8424
     NAL call no. 500 P21P
     Descriptors: prion proteins, NvCreutzfeldt-Jakob-disease, scrapie, polymorphism, disease incubation times, bovine spongiform encephalopathy quantitative trait loci, prion protein gene.

155. Lloyd, T.; American Agricultural Economics Association. The impact of food scares on beef and inter-related meat markets. [S.l. : s.n. 2001?]. "Selected paper, American Agricultural Economics Association annual meeting, Chicago, IL, August 5-August 8, 2001."
     NAL call no. HD9421.4.I47 2001
     Descriptors: meat industry and trade, meat inspection, BSE, Great Britain.

156. Lorenzen, S. BSE ist keine Naturkatastrophe, sondern ein menschengemachtes Problem: Wie lasst es sich in Wurde losen. BSE is not a natural disaster, but a man-made problem: how can the BSE crisis be solved in dignity? Fleischwirtschaft. 2001, 81: 4, 127-133; Many ref. ISSN: 0015-363X. In German with an English summary.
     NAL call no. 280.38 F62
     Descriptors: BSE, bovine spongiform encephalopathy, disease control, public health concerns.

157. Lucker, E.; Horlacher, S.; Eigenbrodt, E. Brain in human nutrition and variant Creutzfeldt-Jakob disease risk (vCJD): detection of brain in retail liver sausages using cholesterol and neuron specific enolase (NSE) as markers. British Journal of Nutrition 2001 Aug. 86 Suppl 1: S115-S119. ISSN: 0007-1145
     Abstract: No information is available about the consumption of brain via meat products. With respect to the new variant of Creutzfeldt-Jakob disease (vCJD) and the presumed food-borne transmission of bovine spongiform encephalopathy (BSE) to humans, a preliminary survey for brain and/or spinal cord (tissues of the central nervous system, CNS) was conducted. We applied a previously developed integrated procedure using cholesterol and neuron specific enolase (NSE) as markers. Quantification of cholesterol had to be backed up by NSE immunochemistry in order to account for low specificity and relatively high variances. Out of 126 high-quality finely graded liver sausages, five samples (4 %) showed positive NSE immunoresponses. In four of these samples a transgression of the normal maximum cholesterol content was obtained. The identification of such a considerable number of CNS-positive sausages indicates that brain consumption is not as rare as previously assumed. Overall, the present integrated method could be successfully applied for the detection of CNS in heat-treated meat products. Its routine application in official food control would deter illegal practice and thus help to control transmissible spongiform encephalopathies.
     NAL call no. 389.8 B773
     Descriptors: detection method, brain material, bovine-based meat products, food screening, contamination control, cholesterol and neuron specific enolase.

158. Lundberg, P. O. Annu bara borjan av galna ko-sjukan? Creutzfeldt-Jakobs sjukdom och andra prionsjukdomar: nulaget. [Still a small problem with the mad cow disease? Creutzfeldt-Jakob disease and other prion diseases: current status] Lakartidningen. 2001 Jan 10. 98(1-2): 19-24 ISSN: 0023-7205. In Swedish.
     Abstract: This review is based on recent published research on the BSE/CJD/vCJD problem mainly from UK, Germany and France. The situation in Sweden seems to be fortunate for several reasons. The use of meat and bonemeal as animal fodder was forbidden in this country 13 years ago. Sweden has not had any sheep with scrapie for many years. No animals with BSE have so far been found in our country. The incidence of sporadic CJD in this country followed retrospectively from 1985 to 1996 and prospectively from 1997 to 1999 has been around 1.2 per million per year with no significant increase. Only few cases of familial CJD are known. No patient with iatrogenic CJD has ever been found. The use of growth hormone derived from human pituitary glands was abandoned in 1985 when recombinant human growth hormone became available. So far there is no indication that any of the CJD cases diagnosed in Sweden has been of the vCJD type, the one linked to BSE. However, as the incubation period for prion diseases is very long and the Swedes are frequent travelers there is a risk that people from our country could have contracted vCJD through consuming meat products in countries with BSE. As a precaution the consumption of brain, spinal cord, lymphatic tissue, lungs, and gastrointestinal tract should be avoided. Human pituitary derived growth hormone is still available in some countries and might be illegally imported into Sweden.
     Descriptors: Review article, BSE, NvCreutzfeldt-Jakob Disease, feed ingredients, incidence, disease free, scrapie, battle, sheep, travel risks.

159. Lundberg, P.O. Far, kor--och slakt? [Sheep, cattle--and slaughtering?] Lakartidningen. 2001 Feb 28. 98(9): 990-991 ISSN: 0023-7205. In Swedish.
     Descriptors: sheep, cattle, slaughtering issues, BSE, scrapie.

160. MacKnight, C. Clinical implications of bovine spongiform encephalopathy. Clinical Infectious Diseases. 2001 Jun 15. 32(12): 1726-1731 ISSN: 1058-4838
     Abstract: Bovine spongiform encephalopathy (BSE) is a new prion disease that was first identified in the United Kingdom in 1987. Its appearance was likely caused by changes in the rendering process used to produce a meat and bone supplement for cattle, changes that allowed this prion to enter the bovine food supply. Despite measures that were made to reduce the risk to humans, a new variant of Creutzfeldt-Jakob disease appeared in the mid-1990s and has been linked to BSE. Although the extent of the disease's impact on humans is not yet known, current estimates predict that there will be 136,000 cases of this fatal disease by the year 2040. The risk to humans of medications produced with bovine materials, gelatin, and blood transfusion is unknown.
     NAL call no. RC111 R4
     Descriptors: BSE, risk to human health, NvCreutzfeldt-Jakob-Disease, UK, disease incidence projections, transmission risks of bovine-based pharmaceuticals and other materials, blood transfusions.

161. Magdzik,W. Encefalopatie gabczaste (choroby prionowe) zwierzat. [Spongiform encephalopathy (prion diseases) in animals] Przeglad Epidemiologiczny . 2001. 55(1 Suppl 2): 71-74. ISSN: 0033-2100. In Polish.
     Descriptors: prion diseases, animal incidence, BSE, scrapie.

162. Maissen, M.; Roeckl, C.; Glatzel, M.; Goldmann, W.; Aguzzi, A. Plasminogen binds to disease-associated prion protein of multiple species. Lancet. 2001 Jun 23. 357(9273): 2026-2028 ISSN: 0140-6736
     NAL call no. 448.8 L22
     Descriptors: prion protein, transmissible spongiform encephalopathies, biochemistry.

163. Mangen, M.J.J.; Burrell, A.M. Decomposing preference shifts for meat and fish in the Netherlands. Journal of Agricultural Economics. 2001, 52: 2, 16-28; 30 ref. ISSN: 0021-857X.
     NAL call no. 281.9 AG8
     Descriptors: beef, BSE, bovine spongiform encephalopathy, consumer preferences, elasticities, fish, meat products, pork, poultry meat, consumer preferences, demand changes, Netherlands.

164. Manolakou, K.; Beaton, J.; McConnell, I.; Farquar, C.; Manson, J.; Hastie, N.D.; Bruce, M.; Jackson, I.J. Genetic and environmental factors modify bovine spongiform encephalopathy incubation period in mice. Proc Natl Acad Sci USA. Washington, D.C. : National Academy of Sciences, June 19, 2001. v. 98 (13) p. 7402-7407. ISSN: 0027-8424
     Abstract: The incubation period (IP) and the neuropathology of transmissible spongiform encephalopathies (TSEs) have been extensively used to distinguish prion isolates (or strains) inoculated into panels of inbred mouse strains. Such studies have shown that the bovine spongiform encephalopathy (BSE) agent is indistinguishable from the agent causing variant Creutzfeldt-Jakob disease (vCJD), but differs from isolates of sporadic CJD, reinforcing the idea that the vCJD epidemic in Britain results from consumption of contaminated beef products. We present a mouse model for genetic and environmental factors that modify the incubation period of BSE cross-species transmission. We have used two mouse strains that carry the same prion protein (PrP) allele, but display a 100-day difference in their mean IP following intracerebral inoculation with primary BSE isolate. We report genetic effects on IP that map to four chromosomal regions, and in addition we find significant factors of host environment, namely the age of the host's mother, the age of the host at infection, and an X-cytoplasm interaction in the host.
     NAL call no. 500 N21P
     Descriptors: mice, bovine spongiform encephalopathy. isolation, cattle brain, prepatent period. duration, alleles, animal proteins, application methods, animal models, man, foodborne diseases, genetic effects, disease transmission, age, haplotypes, major histocompatibility complex. quantitative traits. loci.

165. Marouby, H. La consommation de viandes dans l'Union Europeenne: la situation avant la crise. [The consumption of meat in the European Union: the situation before the crisis.] Techni Porc. 2001, 24: 1, 3-4. ISSN: 0181-6764
     NAL call no. SF391.T4
     Descriptors: meat, food consumption, pork, BSE, bovine spongiform encephalopathy, cattle diseases, prion diseases.

166. Masters, C.L. The emerging European epidemic of variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy: lessons for Australia. Medical Journal of Australia, 2001 Feb 19. 174(4): 160-161 ISSN: 0025-729X
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, zoonotic diseases, health risks, trade concerns, disease control.

167. Mastrangelo, P.; Westaway, D. The prion gene complex encoding PrP(C) and Doppel: insights from mutational analysis. Gene. 2001 Sep 5. 275(1): 1-18 ISSN: 0378-1119
     Abstract: The prion protein gene, Prnp, encodes PrP(Sc), the major structural component of prions, infectious pathogens causing a number of disorders including scrapie and bovine spongiform encephalopathy (or BSE). Missense mutations in the human Prnp gene cause inherited prion diseases such as familial Creutzfeldt-Jakob disease. In uninfected animals Prnp encodes a glycophosphatidylinositol (GPI)-anchored protein denoted PrP(C) and in prion infections PrP(C) is converted to PrP(Sc) by templated refolding. Though Prnp is conserved in mammalian species, attempts to verify interactions of putative PrP binding proteins by genetic means have proven frustrating and the ZrchI and Npu lines of Prnp gene-ablated mice (Prnp(0/0) mice) lacking PrP(C) remain healthy throughout development. This indicates that PrP(C) serves a function that is not apparent in a laboratory setting or that other molecules have overlapping functions. Current possibilities involve shuttling or sequestration of synaptic Cu(II) via binding to N-terminal octapeptide residues and/or signal transduction involving the fyn kinase. A new point of entry into the issue of prion protein function has emerged from identification of a paralogue, Prnd, with 24% coding sequence identity to Prnp. Prnd lies downstream of Prnp and encodes the doppel (Dpl) protein. Like PrP(C), Dpl is presented on the cell surface via a GPI anchor and has three alpha-helices: however, it lacks the conformationally plastic and octapeptide repeat domains present in its well-known relative. Interestingly, Dpl is overexpressed in the Ngsk and Rcm0 lines of Prnp(0/0) mice via intergenic splicing events. These lines of Prnp(0/0) mice exhibit ataxia and apoptosis of cerebellar cells, indicating that ectopic synthesis of Dpl protein is toxic to central nervous system neurons: this inference has now been confirmed by the construction of transgenic mice expressing Dpl under the direct control of the PrP promoter. Remarkably, Dpl-programmed ataxia is rescued by wild-type Prnp transgenes. The interaction between the Prnp and Prnd genes in mouse cerebellar neurons may have a physical correlate in competition between Dpl and PrP(C) within a common biochemical pathway that when mis-regulated leads to apoptosis.
     NAL call no. QH442.A1G4
     Descriptors: PrP, prion protein functions, missence mutations, mouse models (ZrchI, Npu, Ngsk a Rcm0) transgenic mouse model, doppel proteins.

168. Massara, F.; Scavone, M.; Ferraro, A. Infezioni emergenti e sanita pubblica. [Emergent infections and public health.] Obiettivi e Documenti Veterinari. 2001, 22: 1, 61-64. ISSN: 0392-1913. In Italian.
     Descriptors: foodborne zoonotic diseases, BSE, bovine spongiform encephalopathy, Escherichia coli, disease prevention, public health concerns, salmonellosis, listeriosis, toxoplasmosis, disease control and prrevention, disease-transmission, domestic animals, animal based foods and products.

169. Mazzocchi, M.; Heckelei, T. (ed.); Witzke, H.P. (ed.); Henrichsmeyer, W. Econometric methods for evaluating consumer response to food scares: a structural approach. Agricultural sector modelling and policy information systems. Proceedings of the 65^th European Seminar of the European Association of Agricultural Economists EAAE, Bonn, Germany, 29-31-March, 2000. 2001, 111-120; 36 ref. Wissenschaftsverlag Vauk Kiel KG; Kiel; Germany. ISBN: 3-8175-0329-6
     Descriptors: BSE, bovine spongiform encephalopathy, case studies, food demand models demand functions, food consumption, meat, EM algorithm, Kalman filter.

170. Mazzoni, I.E.; Ledebur, H; Cashman, N. Signal transduction mechanisms linked to prion protein. Society for Neuroscience Abstracts. 2001; 27 (1): 112. ISSN: 0190-5295. 31st Annual Meeting of the Society for Neuroscience, San Diego, California, USA, November 10-15, 2001
     NAL call no. QP351.S6
     Descriptors: cortical postmitotic cultures, bse, Creutzfeldt-Jakob disease, PrP knockout mice, concavalin A, calcium flux, tyrosine phosphorylation, splenocytes.

171. McDonnell, K.; Desmond, J.; Leahy, J.J.; Howard, Hildige R.; Ward, S. Behavior of meat and bonemeal/peat pellets in a bench scale fluidised bed combustor. Energy Oxford. 2001, 26: 1, 81-90; 11 ref. ISSN: 0360-5442
     Descriptors: BSE, animal by-product disposal, fluidised bed combustor, combustion tests, pellets with meat and bone meal, alternative energy source.

172. McGill, I. Phillips report and the origin of BSE. Veterinary Record. 2001 Jan 13. 148(2): 60 ISSN: 0042-4900
     NAL call no. 41.8 V641
     Descriptors: Bovine spongiform encephalopathy, etiology, cattle, mutation, nerve tissue proteins, genetics, prions.

173. Mead, Simon; Mahal, Sukhvir P.; Beck, John; Campbell, Tracy; Farrall, Martin; Fisher, Elizabeth; Collinge, John. Sporadic: But not variant: Creutzfeldt-Jakob disease is associated with polymorphisms upstream of PRNP exon 1. American Journal of Human Genetics. December, 2001; 69 (6): 1225-1235. ISSN: 0002-9297.
     NAL call no. QH431.A1A54
     Descriptors: humans, NvCruetzfeldt-Jakob Disease, etiologies, BSE, prions, PrP gene, codon 129, mouse model, 56 polymorphic sites

174. Menges, T.; Langefeld, T.W.; Krumholz, W.; Hempelmann, G. Ubertragbare spongiforme Enzephalopathien--Anaesthesiologisches und intensivmedizinisches Management. [Transmissible spongiform encephalopathies--anesthetics and intensive care management.] Anasthesiologie, Intensivmedizin, Notfallmedizin, Schmerztherapie-AINS. 2001 Feb. 36(2): 79-89 ISSN: 0939-2661. In German.
     Abstract: The transmissible spongiform encephalopathies (TSE) are known to affect humans and various animals. The bovine spongiform encephalopathy (BSE) and the human Creutzfeldt-Jacob disease (CJD) are among the most notable degenerative disorders caused by prions. Considering the BSE epidemic and the description of a new variant of Creutzfeldt-Jacob disease (nvCJD), which is probably related to bovine spongiform encephalopathy, TSE have recently gained a lot of public attention. Although the causative factors (prions, viruses) are still under discussion, none of the present concepts are explanatory for all aspects of the human CJD. CJD may present as a sporadic, genetic, or infectious illness and there is now considerable concern that bovine prions may have been passed to humans. To exclude transmission of CJD via medical products and instruments, the effectiveness of cleaning, disinfection and sterilization procedures must be firmly established. This manuscript presents an overview to anaesthesiology and intensive care medicine of recommended inactivation procedures and assessed these procedures in the light of the inactivation of prions.
     Descriptors: TSE’s, BSE, CJD, NvCreutzfeldt-Jacob Disease, prions, disinfection/inactivations procedures, anesthetic and analgesic equipment.

175. Miele, G.; Manson, J.; Clinton, M. A novel erythroid-specific marker of transmissible spongiform encephalopathies. Nature Medicine. 2001 Mar. 7(3): 361-364 ISSN: 1078-8956
     Abstract: Transmissible spongiform encephalopathies (TSE) are a group of invariably fatal neurodegenerative diseases and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease in deer and elk, and Kuru disease, Creutzfeldt-Jakob disease (CJD) and variant CJD in humans. The pathological effects of disease occur predominantly in the CNS (central nervous system), where common hallmarks include vacuolation, gliosis, accumulation of a protease-resistant, abnormally folded isoform of the prion protein (PrPSc) and neuronal cell death. Lack of understanding of the molecular mechanisms underlying disease pathogenesis, particularly in non-CNS tissues, means that there are currently no effective strategies for early diagnosis or therapeutic intervention of TSEs. Here we report the first identification of a molecular marker that is easily detectable in readily accessible tissues. We demonstrate that a dramatic decrease in expression of a transcript specific to erythroid lineage cells is a common feature of TSEs. Our findings indicate a previously unrecognized role for involvement of the erythroid lineage in the etiology of TSE pathogenesis and should provide a new focus for research into diagnostic and therapeutic strategies.
     Descriptors: transmissible spongiform encephalopathies, molecular marker, pathogenesis, research and diagnostic directions, non-CNS tissues, early disgnosis, prion diseases.

176. Miles, S.; Frewer, L.J. Investigating specific concerns about different food hazards. Food Quality and Preference. 2001, 12: 1, 47-61; 35 ref. ISSN: 0950-3293
     NAL call no. TX367.F66
     Descriptors: food safety, food hazards, consumer attitudes, public health concerns, microbial and pesticide contamination of food, genetic engineered; foods, BSE, bovine spongiform encephalopathy, risk factors, laddering techniques.

177. Murphy, C.; Breen, C.; Rogers, M.; Giese, M. Interferon gamma and prostaglandin in BSE-infected cattle. Cytokine. 2001 Feb 7. 13(3): 169-173 ISSN: 1043-4666
     Abstract: Suspect field cases of BSE infection (displaying clinical signs) were examined for possible alterations of cytokine/autacoid plasma levels and were compared to control cases (not displaying clinical signs of BSE infection). Interferon gamma (IFN-gamma) plasma levels were demonstrated as being elevated in all suspected field cases of BSE infection (irrespective of BSE status; determined via postmortem histopathological examination). We demonstrated that plasma IFN-gamma levels were significantly (P<0.005) higher in suspect cases of BSE infection than in control cases. BSE-positive prostaglandin-E(2), (PGE(2)) plasma levels were demonstrated as being elevated 1.25-fold above BSE-negative cases and 2.22-fold above control cases. No significant (P>0.5) increase in PGE(2)plasma levels was recorded between BSE-positive and -negative. IFN-gamma and PGE(2)plasma levels were examined using commercially available ELISA assay. The results presented in this publication are the first demonstration of alteration in immune state in animals with BSE.
     NAL call no. QR185.C95C986
     Descriptors: bovine spongiform encephalopathy, cattle, IFN-gamma levels, field cases, altered immune status.

178. Murray, G. Feed controls -- stopping BSE (Mad Cow Disease). Agnote NSW Agriculture. 2001, No. DAI-227(1st Ed.), 2 pp. NSW Agriculture; Orange; Australia. ISSN: 1034-6848
     NAL call no. SF55 A8 A36
     Descriptors: BSE, bovine spongiform encephalopathy, cows, feeds, labeling controls, meat and bone meal, prion diseases, control measures.

179. Narang, H.K. A critical review of atypical cerebellum-type Creutzfeldt-Jakob disease: its relationship to "new variant" CJD and bovine spongiform encephalopathy. Experimental Biology and Medicine (Maywood). 2001 Jul. 226(7): 629-639 ISSN: 1535-3702
     Abstract: Shortly after the appearance of bovine spongiform encephalopathy (BSE), Creutzfeldt-Jakob disease (CJD) was identified in young patients with nonclassical presentation such as difficulty in balancing and ataxia. The classical CJD in older patients starts with dementia. To distinguish between the two types, CJD in young persons has been termed "new variant" (nvCJD). The distinguishing features of classical CJD include initial presentation with dementia, confluent spongiform changes are very unusual in the cerebellum, and PrP plaques are rarely observed. For nvCJD, initially, difficulty with balancing and ataxia occurs, confluent spongiform changes are seen in the cerebellum, and a large number of PrP plaques are seen. The Icelandic observation of sheep scrapie revealed a predominantly ataxic form of scrapie, termed Type II, rather than the itchy form termed Type I. Both types have been known to exist in Europe. Since the clinical signs of Type II scrapie in sheep with trembling and ataxia are similar to those seen in BSE and nvCJD, this suggests that Type II is the cause of BSE and nvCJD. Over 8 years, from 1989 to 1996, I examined the clinical histories of 33 CJD cases aged between the ages of 18 and 84. Six under the age of 40 and 15 over the age of 40 had leading clinical features such as difficulty in balancing and ataxia similar to those seen in the young cases classified as "nvCJD." Brains were examined from the six of 15 cases over the age of 40, which revealed similar pathology to that seen in young patients classified as "nvCJD." These findings suggest that all age groups are susceptible to the strain of the agent derived from BSE cattle.
     NAL call no. QH301 E9
     Descriptors: BSE, NvCreutzfeldt-Jakob Disease, young humans, disease comparison, brain tissues, cerebellum.

180. Narang, H.K. Lingering doubts about spongiform encephalopathy and Creutzfeldt-Jakob disease. Experimental Biology and Medicine (Maywood). 2001 Jul. 226(7): 640-652 ISSN: 1535-3702
     Abstract: Bovine spongiform encephalopathy (BSE) is an infectious disease and has been transmitted orally to many other animals, including humans. There is clear evidence of maternal transmission, although disagreement on the source of the BSE agent remains. The current theories link the origin of BSE to common scrapie in sheep. Twenty different strains of the scrapie agent have been isolated from sheep. A search of the literature indicates two distinct clinical syndromes in sheep, both of which have been called scrapie. I have designated these Type I (the common type), which exhibits itchiness and lose their wool, and Type II, which exhibits trembling and ataxia. Sheep inoculated with BSE develop Type II scrapie and they exhibit trembling. When cattle or mink are injected with the Type I strain, only a few will develop a clinical disease. By contrast, no clinical disease has so far been shown in cattle or mink by feeding them with Type I-infected sheep brains. However, either by injecting or feeding with the BSE strain, 100% of calves and mink develop the clinical disease. Evidence suggests that Type II is the cause of BSE. Identical clinical signs of Type II trembling are found in kuru and many of the recent cases of Creutzfeldt-Jakob disease. The BSE agent has caused spongiform encephalopathies (SEs) in domestic cats, tigers, and in some species of ruminants in zoos. The nature of the BSE agent remains unchanged when passaged through a range of species, irrespective of their genetic make up, demonstrating that variations in the host PrP gene are not a major factor in the susceptibility to the BSE agent. Since more than 85 zoo animals of many species have been diagnosed with SEs, from these studies it seems reasonable to conclude that the BSE agent can infect almost all mammalian species, including humans. For eradication of BSE and to reduce the risk of infection to humans, the development of a vaccine against BSE is suggested. Such a possibility should be fully explored.
     NAL call no. QH301 E9
     Descriptors: BSE, scrapie agents, Type I and Type II clinical syndromes, sheep, mink, cattle, Type comparisons, etiology, zoo animals, mammalian susceptibility.

181. Negro, A.; Ballarin, C.; Bertoli, A.; Massimino, M.L.; Sorgato, M.C. The metabolism and imaging in live cells of the bovine prion protein in its native form or carrying single amino acid substitutions. Molecular and Cellular Neurosciences 2001 Mar. 17(3): 521-538. ISSN: 1044-7431
     Abstract: Prion diseases are probably caused by an abnormal form of a cellular glycoprotein, the prion protein. Recent evidence suggests that the prion strain causing BSE has been transmitted to humans, thereby provoking a variant form of Creutzfeldt-Jacob disease. In this work, we analyzed the behavior of normal and malformed isoforms of the bovine PrP in transfected mammalian cell lines. Biochemical and immunocytochemical assays were complimented with imaging of live cells expressing fusion constructs between PrP and GFP. Bovine homologues of human E200K and D178N (129M) mutations were used as models of pathogenic isoforms. We show that the GFP does not impair the metabolism of native and mutant bPrPs and is thus a valid marker of PrP cellular distribution. We also show that each amino acid replacement provokes alterations in the cell sorting and processing of bPrP. These are different from those ascribed to both murine mutant homologues. However, human and bovine PrPs carrying the D178N genotype had similar cellular behavior.
     Descriptors: prion protein, behavior of normal and isoforms, bovine PrP, transfected cell lines, GFP, E200K, D178N (129M).

182. O'-Farrell, K.; Dillon, P.; Mee, J.; Crosse, S.; Nolan, M.; Byrne, N.; Reidy, M.; Flynn, F.; Condon, T. Strategy for restocking of Moorepark after depopulation following bovine spongiform encephalopathy. Irish Veterinary Journal. 2001, 54: 2, 70-75; 5 ref. ISSN: 0368-0762.
     NAL call no. 41.8 IR4
     Descriptors: BSE, bovine spongiform encephalopathy, cattle diseases, animal-health, dairy cattle, disease control, Moorepark Centre Farm restocking strategy, herd health status monitoring.

183. Office International des Epizooties. Foot and mouth disease in Turkey, Peninsular Malaysia and Kazakhstan; Classical swine fever in Spain and Slovakia; Bovine spongiform encephalopathy in Greece. Disease Information Office International des Epizooties. 2001, 14: 27, 165-172. ISSN: 1012-5329
     NAL call no. SF781 D57
     Descriptors: BSE, bovine spongiform encephalopathy, disease incidence statistics, epidemiology, foot and mouth disease, outbreaks, swine fever

184. O'Mara F.; O' Doherty J. The effect of removing fishmeal and meat-and-bone meal from animal diets. Irish Veterinary Journal. 2001, 54: 5, 244-245. ISSN: 0368-0762
     NAL call no. 41.8 IR4
     Descriptors: BSE, animal protein in feeds, alternative replacements, costs, nutritional values, Ireland.

185. Oomkes, C.; van Knapen, F. Cows, cats, and FSE: death penalty justified? Veterinary Quarterly 2001 Jan. 23(1): 51-52 ISSN: 0165-2176
     Abstract: Transmissible spongiform encephalopathies affect a number of mammalian species. The most common spongiform encephalopathies are scrapie in sheep and Bovine Spongiform Encephalopathy (BSE) in cattle. Feline Spongiform Encephalopathy (FSE) is a related disorder in domestic cats. Because of the link between BSE and FSE, cats are put on a par with cattle, in terms of politics and regulations. In the Netherlands, when a case of BSE is found on a farm, not only the ruminants, but also the cats are taken away for post-mortem examination. So far, the cats examined have always been negative for FSE. There are no scientific reasons for destroying the cats on farms where BSE has been found.
     NAL call no. SF601 V46
     Descriptors: BSE, feline spongiform encephalopathy, euthanizing cats, farms positive for BSE, incidence of disease.

186. Operativi gli interventi per fronteggiare la Bse. [Financial interventions to cope with BSE.] Informatore Agrario. 2001, 57: 12, 13. ISSN: 0020-0689. In Italian.
     NAL call no. 281.8 IN32
     Descriptors: bovine spongiform encephalopathy, cattle diseases, nervous system diseases, prevention, prion diseases, support measures.

187. Overgaauw, P. A. BSE en petfood: is diervoeding wel veilig? [BSE and pet food: is animal feed safe?] Tijdschrift voor Diergeneeskunde. 2001 Apr 15. 126(8): 292 ISSN: 0040-7453. In Dutch.
     NAL call no. 41.8 T431
     Descriptors: BSE contaminated pet foods, cats, transmissible spongiform encephalopathies.

188. Pallaroni, Lea; Bjorklund, Erland; von-Holst, Christoph; Unglaub, Wolfgang. Determination of rendering plant sterilization conditions using a commercially available ELISA test kit developed for detection of cooked beef. Journal of AOAC-International. November-December, 2001; 84 (6): 1884-1890. ISSN: 1060-3271.
     NAL call no. S583 A7
     Descriptors: BSE, enzyme-linked immunosorbent assay (ELISA) test kit, cooked beef/pork detection, rendering process, laboratory conditions, temperature, time, particle size and meat composition.

189. Paukstadt, W. BSE-Rinder in Deutschland. Mussen wir bald auch mit vCJK-Patienten rechnen? [BSE-cattle in Germany. Must we soon also count on vCJK patients?] MMW-Fortschritte-der-Medizin. 2001 Jan 25. 143(4): 12-13 ISSN: 1438-3276. In German.
     Descriptors: Creutfeldt-Jakob Syndrome transmission, brain pathology, cattle, diagnosis, Germany, risk factors.

190. Peretz, D.; Williamson, R.A.; Kaneko, K.; Vergara, J.; Leclerc, E.; Schmitt-Ulms, G.; Mehlhorn, I. R.; Legname, G.; Wormald, M. R.; Rudd, P.M.; Dwek, R.A.; Burton, D.R.; Prusiner, S.B. Antibodies inhibit prion propagation and clear cell cultures of prion infectivity. Nature. 2001 Aug 16. 412(6848): 739-743 ISSN: 0028-0836
     Abstract: Prions are the transmissible pathogenic agents responsible for diseases such as scrapie and bovine spongiform encephalopathy. In the favoured model of prion replication, direct interaction between the pathogenic prion protein (PrPSc) template and endogenous cellular prion protein (PrPC) is proposed to drive the formation of nascent infectious prions. Reagents specifically binding either prion-protein conformer may interrupt prion production by inhibiting this interaction. We examined the ability of several recombinant antibody antigen-binding fragments (Fabs) to inhibit prion propagation in cultured mouse neuroblastoma cells (ScN2a) infected with PrPSc. Here we show that antibodies binding cell-surface PrPC inhibit PrPSc formation in a dose-dependent manner. In cells treated with the most potent antibody, Fab D18, prion replication is abolished and pre-existing PrPSc is rapidly cleared, suggesting that this antibody may cure established infection. The potent activity of Fab D18 is associated with its ability to better recognize the total population of PrPC molecules on the cell surface, and with the location of its epitope on PrPC. Our observations support the use of antibodies in the prevention and treatment of prion