USDA logo
United States
Department of
Agriculture

ARS logo
Agricultural
Research
Service

NAL logo
National
Agricultural
Library

AWIC logo
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.

Bibliography of Selected Articles

2004 | 2003 | 2002 | 2001 | 2000 | 1999 | 1998 | 1997 | 1996 | 1995

 

An Introduction to BSE and other TSEs

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.

Note: Also see Special Reference Brief, Bovine Spongiform Encephalopathy by Janice C. Swanson, December 1990, Animal Welfare Information Center.

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

Top of Document | Bibliography

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.

Top of Document | Bibliography


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.