Prusiner S B
Department of Neurology, University of California, San Francisco, CA 94143, USA.
Science. 1997 Oct 10;278(5336):245-51. doi: 10.1126/science.278.5336.245.
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.
牛海绵状脑病（BSE）和人类克雅氏病（CJD）是由朊病毒引起的最著名的中枢神经系统退行性疾病。CJD 可能表现为散发性、遗传性或传染性疾病。朊病毒是不含核酸的可传播颗粒，似乎仅由一种修饰蛋白（PrPSc）组成。正常的细胞朊蛋白（PrPC）通过翻译后过程转化为 PrPSc，在此过程中它获得了高β-折叠含量。人们认为 BSE 是同类相食的结果，其中错误的工业做法生产了受朊病毒污染的牛饲料。现在人们非常担心牛朊病毒可能已传播给人类，导致一种新形式的 CJD。
