Mechanisms of disease - Insights into prion strains and neurotoxicity

被引:261
作者
Aguzzi, Adriano [1 ]
Heikenwalder, Mathias [1 ]
Polymenidou, Magdalini [1 ]
机构
[1] Univ Zurich Hosp, Inst Neuropathol, CH-8091 Zurich, Switzerland
关键词
D O I
10.1038/nrm2204
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Transmissible spongiform encephalopathies ( TSEs) are neurodegenerative diseases that are caused by prions and affect humans and many animal species. It is now widely accepted that the infectious agent that causes TSEs is PrPSc, an aggregated moiety of the host-derived membrane glycolipoprotein PrPC. Although PrPC is encoded by the host genome, prions themselves encipher many phenotypic TSE variants, known as prion strains. Prion strains are TSE isolates that, after inoculation into distinct hosts, cause disease with consistent characteristics, such as incubation period, distinct patterns of PrPSc distribution and spongiosis and relative severity of the spongiform changes in the brain. The existence of such strains poses a fascinating challenge to prion research.
引用
收藏
页码:552 / 561
页数:10
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