The prion's elusive reason for being

被引：331

作者：

Aguzzi, Adriano ^{[1
]}

Baumann, Frank ^{[1
]}

Bremer, Jullane ^{[1
]}

机构：

[1] Univ Zurich, Inst Neuropathol, CH-8091 Zurich, Switzerland

来源：

ANNUAL REVIEW OF NEUROSCIENCE | 2008年 / 31卷

关键词：

transmissible spongiform encephalopathy; prion diseases; PrPC; PrPSc;

D O I：

10.1146/annurev.neuro.31.060407.125620

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

The protein-only hypothesis posits that the infectious agent causing transmissible spongiform encephalopathies consists of protein and lacks any informational nucleic acids. This agent, termed prion by Stanley Prusiner, is thought to consist partly of PrPSc, a conformational isoform of a normal cellular protein termed PrPC. Scientists and Jay persons have been fascinated by the prion concept, and it has been subjected to passionate critique and intense experimental scrutiny. As a result, PrPC and its isoforms rank among the most intensively studied proteins encoded by the mammalian genome. Despite all this research, both the physiological function of PrPC and the molecular pathways leading to neurodegeneration in prion disease remain unknown. Here we review the salient traits of those diseases ascribed to improper behavior of the prion protein and highlight how the physiological functions of PrPC may help explain the toxic phenotypes observed in prion disease.

引用

页码：439 / 477

页数：39

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