Prion Diseases and the BSE Crisis
- 10 October 1997
- journal article
- review article
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 278 (5336) , 245-251
- https://doi.org/10.1126/science.278.5336.245
Abstract
Bovine spongiform encephalopathy (BSE) and human Creutzfeldt-Jakob disease (CJD) are among the most notable central nervous system degenerative disorders caused by prions. CJD may present as a sporadic, genetic, or infectious illness. Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrP Sc ). The normal, cellular prion protein (PrP C ) is converted into PrP Sc through a posttranslational process during which it acquires a high β-sheet content. It is thought that BSE is a result of cannibalism in which faulty industrial practices produced prion-contaminated feed for cattle. There is now considerable concern that bovine prions may have been passed to humans, resulting in a new form of CJD.Keywords
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