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Biology and Genetics - Bovine Spongiform Encephalopathy (BSE)

BSE is a fatal disease of cows, which is caused by a prion and spread by infected recycled cattle feed. BSE is also the likely cause of neurodegenerative disease in a small number of human cases. 

Bovine spongiform encephalopathy (BSE), more commonly known as mad cow disease, is a fatal neurodegenerative disease that affects the central nervous system of cattle. The current belief is that the primary means of BSE transmission is through cattle eating feed contaminated by the rendered material from other BSE-infected cattle. BSE is caused by infectious agents called prions, which are misfolded proteins. These prions induce brain cell death and produce ‘spongy’ gaps in the brain. In cows, the incubation time from infection until clinical onset of the disease is on average 5 years. The majority of BSE cases have occurred in the United Kingdom, but the disease has also affected other European and non-European countries around the world. Currently, no test is available to detect BSE in live cattle and there is no known treatment for the disease. BSE is an important public health issue because it may be transmitted to humans through consumption of contaminated beef products. The human form of the disease, known as variant Creutzfeldt-Jakob disease (vCJD), is also currently untreatable. Important BSE risk management strategies include banning animal protein from cows, sheep and other ruminants in animal feed and the removal of specified risk materials (SRMs) known to carry a high risk of BSE. 

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Contributor: Roxanne Lewis

Last reviewed: June 2, 2010



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