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Prion Biology Study

Abstract : In the early 1990's a novel disease of cattle was first recognized. This disease, called Bovine Spongiform Encephalopathy (BSE), is a progressive neurological disorder characterized by erratie behavior and pronounced clumsiness in infected animals. Although the disease kills infected animals within a few months after symptoms appear, the appearance of symptoms can take several years. The BSE outbreak led to the destruction of thousands of herds of cattle and the collapse of the British beef industry. The US cattle industry, with over $50 billion in annual sales a year, would be ruined if BSE were diagnosed in US herds. The discovery of BSE was followed by the recognition of novel neurological diseases in several species, including mankind. Since 1996, over 100 humans in the European Union have been diagnosed with a previously undiagnosed neurological disorder, called variant Cruetzfeldt-Jakob disease (vCJD). This newly emerged disease is strikingly similar to BSE in that its victims show symptoms after a long incubation period (estimated to be approximately 10 years) and die after progressive loss of brain function. It is suspected that cattle and people became infected via eating foodstuffs contaminated with infected brain tissue.