Prion Diseases and the BSE Crisis

作者：Stanley B. Prusiner · 发表于：Science · 年份：1997 · DOI：10.1126/science.278.5336.245 · 被引用次数：962 · 研究领域：Prion Diseases and Protein Misfolding、Neurological diseases and metabolism

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 (PrPSc). The normal, cellular prion protein (PrPC) is converted into PrPSc through a posttranslational process during which it acquires a high beta-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.