朊病毒的结构：足以解释朊病毒疾病的多种表型吗？
The structure of prion: is it enough for interpreting the diverse phenotypes of prion diseases?
机构信息
State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing 102206, China.
出版信息
Acta Biochim Biophys Sin (Shanghai). 2013 Jun;45(6):429-34. doi: 10.1093/abbs/gmt021. Epub 2013 Mar 3.
Prion diseases, or transmissible spongiform encephalopathies, are neurodegenerative diseases, which affect human and many species of animals with 100% fatality rate. The most accepted etiology for prion disease is 'prion', which arises from the conversion from cellular PrP(C) to the pathological PrP(Sc). This review discussed the characteristic structure of PrP, including PRNP gene, PrP(C), PrP(Sc), PrP amyloid, and prion strains.
朊病毒病，又称传染性海绵状脑病，是一种神经退行性疾病，可感染人类和多种动物，致死率为 100%。朊病毒病最被认可的病因是“朊病毒”，它源于细胞朊蛋白（PrPC）向病理性朊蛋白（PrPSc）的转化。本综述讨论了朊蛋白的特征结构，包括 PRNP 基因、PrPC、PrPSc、PrP 淀粉样蛋白和朊病毒株。
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