De novo generation of prion strains.

De novo generation of prion strains.
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DOI:
10.1038/nrmicro2650
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发表时间:
2011-09-26
期刊:
Nature reviews. Microbiology
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其他
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朊病毒是一种自我复制的蛋白质,会导致神经退行性疾病,如“疯牛病”。这些错误加工的蛋白质构象在中枢神经系统中积累,导致大脑发生海绵状变化,最终导致死亡。自纯蛋白质假说(假定错误折叠的蛋白质是导致这些疾病的感染因子)成立以来,研究人员试图在实验室中从确定的组分中产生感染性蛋白质,已经取得了不同程度的成功。在这里,我们讨论了最近的几项研究,已经产生了一系列新的朊病毒株在体外表现出越来越高的感染性滴度。这些进展使我们对朊病毒的结构以及它们产生和传播的机制有了前所未有的了解。
Prions are self-replicating proteins that cause neurodegenerative disorders such as “mad cow” disease. These misprocessed protein conformations accumulate in the central nervous system, causing spongiform changes in the brain and eventually death. Since the inception of the protein-only hypothesis — which posits that misfolded proteins are the infectious agents that cause these diseases — researchers seeking to generate infectious proteins from defined components in the laboratory have had varying degrees of success. Here, we discuss several recent studies that have produced an array of novel prion strains in vitro that exhibit increasingly high titers of infectivity. These advances promise unprecedented insight into the structure of prions and the mechanisms by which they originate and propagate.
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