Two alternative pathways for generating transmissible prion disease de novo

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• 作者：Natallia Makarava ; Regina Savtchenko…
• 刊名：Acta Neuropathologica Communications
• 出版年：2015
• 出版时间：December 2015
• 年：2015
• 卷：3
• 期：1
• 全文大小：3,114 KB
• 刊物主题：Neurosciences;
• 出版者：BioMed Central
• ISSN：2051-5960

文摘

Introduction Previous studies established that prion disease with unique strain-specific phenotypes could be induced by in vitro-formed recombinant PrP (rPrP) fibrils with structures different from that of authentic prions, or PrPSc. To explain the etiology of prion diseases, new mechanism proposed that in animals the transition from rPrP fibrils to PrPSc consists of two main steps: the first involves fibril-induced formation of atypical PrPres, a self-replicating but clinically silent state, and the second consists of atypical PrPres-dependent formation of PrPSc via rare deformed templating events.