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Bovine Spongiform Encephalopathy (BSE) and Other Animal Related Transmissible Spongiform EncephalopathiesMarch 2001 (Revised January 2004)AWIC Series #2001-01Updates Bovine Spongiform Encephalopathy, 1990
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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
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.
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.
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.
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.
Alexandru, N. Detectia PrPSC in trunchiul cerebral la
doua ovine in
NAL call no.: SF604.R48
Descriptors: ewes, sheep, natural
scrapie diagnosed, prion diseases, brain stem samples by foramen magnum rapid
method, immunohistochemistry, Romania.
Belay, Ermias D.;
Maddox, Ryan A.; Gambetti, Pierluigi; Schonberger, Lawrence B. Monitoring the occurrence of emerging
forms of Creutzfeldt-Jakob disease in the
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.
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
Bozzetta, E.;
Caramelli, M.; Casalone, C.; Acutis, P.L.; Ru, G. BSE surveillance in
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.
Bosques, Carlos J.;
Imperiali, Barbara. The interplay of
glycosylation and disulfide formation influences fibrillization in a prion
protein fragment. Proceedings of the
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.
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.
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.
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.
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.
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.
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,
NAL call
no.: 381 Am33Pa
Descriptors: BSE, bovine
spongiform encephalopathy, diagnosis, prion-disease, transmissible spongiform
encephalopathy, variant Creutzfeldt-Jakob disease, vCJD.
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.
Chaala, A.; Roy, C. Recycling of meat and bone meal animal
feed by vacuum pyrolysis. Environmental Science and
Technology.
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.
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.
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.
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.
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,
NAL call no.: RC321.N437
Descriptors: elk, deer, chronic
wasting disease, human health risks, prion disease, transmissible spongiform
encephalopathy, meat product, abstract.
Dahlanuddin; Van, Tien
Dam; Liang, J.B.;
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,
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.
Daude, Nathalie;
Marella, Mathieu; Chabry, Joelle. Specific inhibition of pathological prion
protein accumulation by small interfering RNAs. Journal of Cell Science.
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.
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.
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.
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.
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.
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
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.
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.
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.
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.
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.
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.
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.
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.
NAL call no.: 41.8 T445
Descriptors: zoonotic disease,
food safety, role of veterinarians, prion disease surveillance.
Ghani, Azra C.;
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.
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.
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,
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
Gubbins, Simon;
Simmons, Marion M.; Sivam, Kumar; Webb, Cerian R.; Hoinville, Linda J. Prevalence of scrapie infection in
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%).
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.
Heim, D.; Kihm, U. Risk management of transsmissible
spongiform encephalopathies in
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.
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.
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.
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.
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.
Hlasny, J. Nektere poznatky z Britanie o historii
vyzkumu deficitu horciku u prezvykavcu a BSE. [Some information from
Descriptors: cattle, ruminants,
BSE, prion diseases, magnesium, mineral deficiencies,
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.
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.
Ironside, James W. The spectrum of safety: Variant
Creutzfeldt-Jakob disease in the
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,
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.
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.
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.
Kellar, J.A.; Lees,
V.W. Risk management of the
transmissible spongiform encephalopathies in
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.
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.
Descriptors: prion protein,
cattle, CJD, scrapie, TSE, scrapie, prion diseases, pathogenesis.
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.
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,
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,
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.
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.
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.
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.
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.
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.
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.
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.
Lucassen, Ralf;
Nishina, Koren; Supattapone, Surachai. In vitro
amplification of protease-resistant prion protein requires free sulfhydryl
groups. Biochemistry
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.
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.
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.
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.
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.
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
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.
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.
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.
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,
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.
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,
Descriptors: anti-prion substance,
Congo Red, prion disease, drug screening system, cell
culture model, histology and cytology techniques.
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.