与朊蛋白一起探秘：动物的朊病毒疾病

On safari with PrP: prion diseases of animals.

作者信息

Westaway D, Carlson G A, Prusiner S B

机构信息

Dept of Neurology, University of California, San Francisco 94143, USA.

出版信息

Trends Microbiol. 1995 Apr;3(4):141-7. doi: 10.1016/s0966-842x(00)88903-9.

DOI:10.1016/s0966-842x(00)88903-9

PMID:7613755

Abstract

Prions are infectious pathogens that cause fatal neurodegeneration in humans and animals and are composed largely, or entirely, of an aberrant isoform of the host-encoded prion protein (PrP). A post-translational process involving a conformational change in PrP is a significant feature of their replication. Differences in PrP sequences modify the incubation times, neuropathology and properties of prion 'strains'.

摘要

朊病毒是一种传染性病原体，可导致人类和动物发生致命的神经退行性变，其主要或完全由宿主编码的朊病毒蛋白（PrP）的异常异构体组成。涉及PrP构象变化的翻译后过程是其复制的一个重要特征。PrP序列的差异会改变朊病毒“毒株”的潜伏期、神经病理学和特性。