Prion: the chameleon protein

被引:21
|
作者
Zou, W. Q. [1 ,2 ]
Gambetti, P. [1 ,2 ]
机构
[1] Case Western Reserve Univ, Dept Pathol, Cleveland, OH 44106 USA
[2] Case Western Reserve Univ, Natl Prion Dis Pathol Surveillance Ctr, Cleveland, OH 44106 USA
关键词
prion; prion protein; prion diseases; Creutzfeldt-Jakob disease; Gerstmann-Straussler-Scheinker syndrome; Alzheimer's disease; neurodegenerative disorders;
D O I
10.1007/s00018-007-7380-8
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
From Creutzfeldt-Jakob disease (CJD) to variant CJD through Gerstmann-Straussler-Scheinker syndrome, kuru and fatal familial insomnia, the journey leading to current understanding of the basic aspects of human prion diseases has been full of unexpected, but often dramatic and always fascinating twists. Recent progress in modeling prion diseases and characterization of the various prion protein forms reveal that such a wide spectrum of the diseases is associated with the chameleon-like conformational features of prions.
引用
收藏
页码:3266 / 3270
页数:5
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