Prion diseases of humans and animals

被引:28
作者
Prusiner, SB
Telling, G
Cohen, FE
DeArmond, SJ
机构
[1] UNIV CALIF SAN FRANCISCO, DEPT BIOCHEM & BIOPHYS, SAN FRANCISCO, CA 94143 USA
[2] UNIV CALIF SAN FRANCISCO, DEPT CELLULAR & MOL PHARMACOL, SAN FRANCISCO, CA 94143 USA
[3] UNIV CALIF SAN FRANCISCO, DEPT PATHOL, SAN FRANCISCO, CA 94143 USA
来源
SEMINARS IN VIROLOGY | 1996年 / 7卷 / 03期
基金
美国国家卫生研究院;
关键词
neurodegeneration; prion protein; protein conformation; protein X; scrapie;
D O I
10.1006/smvy.1996.0021
中图分类号
Q93 [微生物学];
学科分类号
071005 ; 100705 ;
摘要
Prions cause infectious and genetic neurodegenerative diseases. Transmissible prion particles are composed largely, if not entirely, of an abnormal isoform of the prion protein (PrPSc), which is encoded by a chromosomal gene. A post-translational process involving a profound conformational change converts the cellular prion protein. (PrPC) into PrPSc. PrPC has a high alpha-helix content and is devoid of beta-sheet; whereas, PrPSc has a lower alpha-helix content but is high in beta-sheet. Transgenetic studies argue that a factor(s) designated protein X functions in the formation of PrPSc, perhaps as a molecular chaperone. Mutations in the PrP gene are genetically linked to development of neurodegeneration in humans. These mutations may cause disease by destabilizing one or more of the alpha-helices of PrPC. Investigations of prion diseases may give insights into the more common neurodegenerative diseases.
引用
收藏
页码:159 / 173
页数:15
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