Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, is a transmissible spongiform encephalopathy and fatal neurodegenerative disease in cattle that may be passed to humans who have eaten infected flesh. BSE causes a spongiform degeneration of the brain and spinal cord. BSE has a long incubation period, of 2.5 to 5 years, usually affecting adult cattle at a peak age onset of four to five years. BSE is caused by a misfolded protein—a prion. In the United Kingdom, more than 180,000 cattle were infected and 4.4 million slaughtered during the eradication program. According to statistical estimates made in the 2006, 44,800 French cattle were infected by BSE between 1987 and 1997.
The disease may be most frequently transmitted to humans by eating food contaminated with the brain, spinal cord, or digestive tract of infected carcasses. However, the infectious agent, although most highly concentrated in nervous tissue, can be found in virtually all tissues.
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