HUMAN PRION DISEASES

被引:212
作者
PRUSINER, SB
HSIAO, KK
机构
[1] UNIV CALIF SAN FRANCISCO, DEPT BIOCHEM & BIOPHYS, SAN FRANCISCO, CA 94143 USA
[2] UNIV MINNESOTA, DEPT NEUROL, MINNEAPOLIS, MN 55455 USA
关键词
D O I
10.1002/ana.410350404
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
The prion diseases, sometimes referred to as the ''transmissible spongiform encephalopathies,'' include kuru, Creutzfeldt-Jakob disease, and Gerstmann-Straussler-Scheinker disease of humans as well as scrapie and bovine spongiform encephalopathy of animals. Por many years, the prion diseases were thought to be caused by viruses despite intriguing evidence to the contrary. The unique characteristic common to all of these disorders, whether sporadic, dominantly inherited, or acquired by infection, is that they involve the aberrant metabolism of the prion protein (PrP). In many cases, the cellular prion protein is converted into the scrapie isoform by a posttranslatioflal process that involves a conformational change. Often, the human prion diseases are transmissible to experimental animals and all of the inherited prion diseases segregate with PrP gene mutations.
引用
收藏
页码:385 / 395
页数:11
相关论文
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