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Prion Disease Agent Causes Heart Damage

infected wild mice, which typically die after about 150 days, the engineered mice regularly lived for more than 600 days with minimal symptoms, ultimately dying of old age.

In prion diseases, the normal form of the prion protein is converted into an abnormal, misfolded form. Disease occurs because these abnormal proteins have the ability to convert normal prion proteins into the abnormal form. Like bad apples spoiling the lot, the infectious prions will multiply, and their insolubility will lead to their aggregation and the formation of plaques, which can interfere with normal function in the brain.

Of the prion diseases, most in the news has been bovine spongiform encephalopathy, or mad cow disease, which has caused widespread public concern over the last two decades after it has appeared primarily in cattle in England but also in other countries in Europe, Canada, and the United States. Perhaps the number one reason why a disease that infects cows is of such concern to world governments is that scientists believe that the disease can be transmitted across species through the consumption of tainted meat from a diseased animal's central nervous system. The first major outbreak of mad cow disease in Britain is believed to have originated with the now outlawed practice of feeding cattle meat and bone meal derived from other slaughtered animals. Chronic wasting disease in U.S. deer and elk is also of concern, as the abnormal protein has been found in both wild and farmed animals.

Scientists now know that humans who eat meat from BSE-infected cattle may be susceptible to the prion disease new variant Creutzfeldt-Jakob. There are now more than 140 such cases. This incurable disease is named after a similar condition called Creutzfeldt-Jakob disease, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob, who first diagnosed it. Creutzfeldt-Jakob disease most commonly strikes older people, and it causes neurologic
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