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Classic image of a cow with BSE. A feature of such disease is the inability of the infected animal to
stand.
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Infectious agent
Microscopic "holes" of tissue sections are examined in the lab. Source: APHIS
The infectious agent in BSE is believed to be a specific type of misfolded protein called a prion.
Those prion proteins carry the disease between individuals and cause deterioration of the brain.
BSE is a type of transmissible spongiform encephalopathy (TSE).[10] TSEs can arise in animals
that carry an allele which causes previously normal protein molecules to contort by themselves from
an alpha helical arrangement to a beta pleated sheet, which is the disease-causing shape for the
particular protein. Transmission can occur when healthy animals come in contact with tainted
tissues from others with the disease. In the brain these proteins cause native cellular prion protein to
deform into the infectious state, which then goes on to deform further prion protein in an
exponential cascade. This results in protein aggregates, which then form dense plaque fibers,
leading to the microscopic appearance of "holes" in the brain, degeneration of physical and mental
abilities, and ultimately death.
Different hypotheses exist for the origin of prion proteins in cattle. Two leading hypotheses suggest
that it may have jumped species from the disease scrapie in sheep, or that it evolved from a
spontaneous form of "mad cow disease" that has been seen occasionally in cattle for many
centuries.[11] Publius Flavius Vegetius Renatus records cases of a disease with similar
characteristics in the 4th and 5th Century AD.[12] The British Government enquiry took the view
the cause was not scrapie as had originally been postulated, and was some event in the 1970s that it
was not possible to identify.[13]
Findings published in PLoS Pathogens (September 12, 2008) suggest that mad cow disease also is
caused by a genetic mutation within a gene called Prion Protein Gene. The research shows, for the
first time, that a 10-year-old cow from Alabama with an atypical form of bovine spongiform
encephalopathy had the same type of prion protein gene mutation as found in human patients with
the genetic form of Creutzfeldt-Jakob disease, also called genetic CJD for short. Besides having a
genetic origin, other human forms of prion diseases can be sporadic, as in sporadic CJD, as well as
foodborne. That is, they are contracted when people eat products contaminated with mad cow
disease. This form of Creutzfeldt-Jakob disease is called variant CJD.[14]
Not all scientists agree that the danger of contracting the disease warrants taking extreme measures.
They stress that human infection by mad cow disease has been statistically very small.[citation
needed]
Regulatory failures
In February 2001, the USGAO reported that the FDA, which is responsible for regulating feed, had
not adequately policed the various bans.[25] Compliance with the regulations was shown to be
extremely poor before the discovery of the Washington cow, but industry representatives report that
compliance is now 100%. Even so, critics call the partial prohibitions insufficient. Indeed, US meat
producer Creekstone Farms alleges that the USDA is preventing BSE testing from being conducted.
[26]
The USDA has issued recalls of beef supplies that involved introduction of downer cows into the
food supply. Westland/Hallmark was found to have used electric shocks to prod downer cows into
the slaughtering system in 2007.[27] Possibly due to pressure from large agribusiness, the United
States has drastically cut back on the number of cows inspected for BSE.[28]
See also
• Meat on the bone
• US beef imports in Japan
References
1. ^ "A Focus on Bovine Spongiform Encephalopathy". Pathogens and Contaminants. Food
Safety Research Information Office. November 2007.
http://fsrio.nal.usda.gov/document_fsheet.php?product_id=169. Retrieved 2008-04-07.
2. ^ Brown, David (2001-06-19). "The 'recipe for disaster' that killed 80 and left a £5bn bill".
The Daily Telegraph. http://www.telegraph.co.uk/news/uknews/1371964/The-recipe-for-
disaster-that-killed-80-and-left-a-5bn-bill.html. Retrieved 2008-04-07.
3. ^ a b "Commonly Asked Questions About BSE in Products Regulated by FDA's Center for
Food Safety and Applied Nutrition (CFSAN)". Center for Food Safety and Applied
Nutrition, Food and Drug Administration. 2005-09-14.
http://vm.cfsan.fda.gov/~comm/bsefaq.html. Retrieved 2008-04-08.
4. ^ a b c d e f g h i j k l m "Variant Creutzfeld-Jakob Disease, Current Data (October 2009)". The
National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU), University of Edinburgh.
October 2009. http://www.cjd.ed.ac.uk/vcjdworld.htm. Retrieved 2000-10-14.
5. ^ "Creutzfeldt-Jakob Disease Fact Sheet". National Institute of Neurological Disorders and
Stroke. 2008-02-13. http://www.ninds.nih.gov/disorders/cjd/detail_cjd.htm. Retrieved 2008-
04-08.
6. ^ Valleron AJ, Boelle PY, Will R, Cesbron JY (November 2001). "Estimation of epidemic
size and incubation time based on age characteristics of vCJD in the United Kingdom".
Science (New York, N.Y.) 294 (5547): 1726–8. doi:10.1126/science.1066838. PMID
11721058. Lay summary – BBC News (2001-11-23).
7. ^ a b c "BSE: Disease control & eradication - Causes of BSE", Department for Environment,
Food, and Rural Affairs, March 2007.
8. ^ "The BSE Inquiry", led by Lord Phillips of Worth Matravers, report published October
2000.
9. ^ Harden, Blaine (2003-12-28). "Supplements used in factory farming can spread disease".
The Washington Post. http://archives.seattletimes.nwsource.com/cgi-
bin/texis.cgi/web/vortex/display?slug=madcowdairy28&date=20031228. Retrieved 2008-
04-08.
10. ^ "Bovine Spongiform Encephalopaphy: An Overview" (PDF). Animal and Plant
Health Inspection Service, United States Department of Agriculture. December 2006.
http://www.aphis.usda.gov/publications/animal_health/content/printable_version/BSEbrochu
re12-2006.pdf. Retrieved 2008-04-08.
11. ^ MacKenzie, Debora (17 March 2007). "New twist in tale of BSE's beginnings".
New Scientist 193 (2595): 11. doi:10.1016/S0262-4079(07)60642-3.
http://www.newscientist.com/article/mg19325954.400-new-twist-in-tale-of-bses-
beginnings.html. Retrieved 2009-06-20.
12. ^ Digesta Artis Mulomedicinae, Publius Flavius Vegetius Renatus
13. ^ Vol.1 - Executive Summary of the Report of the Inquiry
14. ^ Richt JA, Hall SM (2008). "BSE case associated with prion protein gene
mutation". PLoS Pathogens 4 (9): e1000156. doi:10.1371/journal.ppat.1000156. PMID
18787697. Lay summary – Newswise (2008-09-12).
15. ^ Vol.2 - 3. The nature and cause of BSE
16. ^ "Mad cow disease: Still a concern". MayoClinic.com. CNN. 2006-02-10.
http://premium.edition.cnn.com/HEALTH/library/ID/00012.html. Retrieved 2009-06-20.
17. ^ "Bovine Spongiform Encephalopathy - "Mad Cow Disease"". Fact Sheets. Food
Safety and Inspection Service. March 2005.
http://www.fsis.usda.gov/Fact_Sheets/Bovine_Spongiform_Encephalopathy_Mad_Cow_Dis
ease/index.asp. Retrieved 2008-04-08.
18. ^ Colchester AC, Colchester NT (2005). "The origin of bovine spongiform
encephalopathy: the human prion disease hypothesis". Lancet 366 (9488): 856–61.
doi:10.1016/S0140-6736(05)67218-2. PMID 16139661.
19. ^ Mago, Chandrika; Sinha, Kounteya (2005-09-02). "India dismisses Lancet's mad
cow". The Times of India. http://timesofindia.indiatimes.com/articleshow/1218869.cms.
Retrieved 2009-06-20.
20. ^ Thompson, Geoff (2005-09-05). "New theory traces mad cow disease to animal
feed exported from India". The World Today (ABC).
http://www.abc.net.au/worldtoday/content/2005/s1453543.htm. Retrieved June 20, 2009.
21. ^ Colchester, A; Colchester, N (28 January 2006). "Origin of bovine spongiform
encephalopathy — Authors' reply". The Lancet (Elsevier) 367 (9507): 298–299.
doi:10.1016/S0140-6736(06)68062-8.
22. ^ Rampton, Sheldon; Stauber, John (2004). Mad Cow USA (1st ed.). Monroe, Maine:
Common Courage Press. ISBN 978-1-56751-110-9.
23. ^ The term "chicken litter" also includes spilled chicken feed as well as fecal matter
and feathers. It is still legal in the United States to use ruminant protein to feed chickens.
Thus, ruminant protein can get into the food chain of cattle in this round about way.
24. ^ Peck, Clint (2004-11-05). "Reader Questions Poultry Litter And "Downer" Bans".
Organic Consumers Association.
http://www.organicconsumers.org/madcow/cow110504.cfm. Retrieved 2009-06-20.
25. ^ Phillips, Kyra; Meserve, Jeanne (2001-02-26). "FDA Not Doing Enough to Prevent
Mad Cow Disease?". Organic Consumers Association.
http://www.organicconsumers.org/madcow/cnn22602.cfm. Retrieved 2009-06-20.
26. ^ 3buddies (2007-05-30). "Creekstone Farms response to USDA appeal of summary
judgement". Press release. Archived from the original on 2007-09-28.
http://web.archive.org/web/20070928064710/http://www.3buddies.com/creekstone/news-
appeal-response.html. Retrieved 2009-06-20.
27. ^ Seltzer, Molly (2008-07-12). "Meat Recalls to Name Retailers". Bloomberg News.
The Washington Post. http://www.washingtonpost.com/wp-
dyn/content/article/2008/07/11/AR2008071102818.html. Retrieved 2009-06-20.
28. ^ "Mad cow watch goes blind". USA Today. 2006-08-03.
http://www.usatoday.com/news/opinion/editorials/2006-08-03-our-view_x.htm. Retrieved
June 20, 2009.
29. ^ "Statistics". Trade Library. U.S. Meat Export Federation.
http://www.usmef.org/TradeLibrary/Statistics.asp. Retrieved 2009-06-20.
30. ^ Weiss, Rick (2007-01-01). "Scientists Announce Mad Cow Breakthrough". The
Washington Post. http://www.washingtonpost.com/wp-
dyn/content/article/2006/12/31/AR2006123100672.html. Retrieved 2007-01-01.
31. ^ "BSE in Belgium". 2006-11-12.
http://home.hetnet.nl/~mad.cow/landen/Belgium.htm. Retrieved 2008-11-09.
32. ^ a b "BSE Cases in North America, by Year and Country of Death, 1993-2008".
Centers for Disease Control and Prevention, Department of Health and Human Services.
2008. http://www.cdc.gov/ncidod/dvrd/bse/images/bse_cases_namerica_2008.gif. Retrieved
2008-11-09.
33. ^ "BSE Positive Findings in the Czech Republic" (pdf). State Veterinary
Administration, Ministry of Agriculture, Czech Republic. 2007. p. 2.
http://www.svscr.cz/files/bse/en_mappositivefindingsbse.pdf. Retrieved 2008-11-09.
34. ^ "The Current Status of BSE and scrapie in Denmark" (pdf). Danish Veterinary and
Food Administration. May 2007.
http://www.uk.foedevarestyrelsen.dk/NR/rdonlyres/ewjvkzzucvuygkq42gxj6umioovybp7ro2
oirh77lz4kacv72gwfhuv6gmt4g643hvuhg4t2wgmynpbco2zpem2ucte/StatusreportonBSEan
dscrapieinDenmarkMay20071.pdf. Retrieved 2008-11-09.
35. ^ France reports more than 900 BSE cases
36. ^ "BSE in Greece". http://home.hetnet.nl/~mad.cow/landen/Greece.htm. Retrieved
2008-11-09.
37. ^ "Israel: BSE testing according to source of cattle and age groups, 2002-2008".
2008-03-05. http://agri3.huji.ac.il/~yakobson/bse/bse_test.htm.
38. ^ "BSE cases - Italy 2001 - 2006". 2006. http://www.izsto.it/ceafoc_bse.htm.
Retrieved 2008-11-10.
39. ^ "Overzicht BSE-gevallen" (in Dutch). Ministerie van Landbouw, Natuur en
Voedselkwaliteit. 2008. http://www.minlnv.nl/portal/page?
_pageid=116,1640440&_dad=portal&_schema=PORTAL&p_document_id=110016&p_nod
e_id=1161644&p_mode=BROWSE. Retrieved 2008-11-09.
40. ^ The number of BSE cases is not available for Thailand.