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The prion's elusive reason for being.
Annual Review of Neuroscience ( IF 13.9 ) Pub Date : 2008-06-19 , DOI: 10.1146/annurev.neuro.31.060407.125620 Adriano Aguzzi 1 , Frank Baumann , Juliane Bremer
Annual Review of Neuroscience ( IF 13.9 ) Pub Date : 2008-06-19 , DOI: 10.1146/annurev.neuro.31.060407.125620 Adriano Aguzzi 1 , Frank Baumann , Juliane Bremer
Affiliation
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 PrP(Sc), a conformational isoform of a normal cellular protein termed PrP(C). Scientists and lay persons have been fascinated by the prion concept, and it has been subjected to passionate critique and intense experimental scrutiny. As a result, PrP(C) and its isoforms rank among the most intensively studied proteins encoded by the mammalian genome. Despite all this research, both the physiological function of PrP(C) 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 PrP(C) may help explain the toxic phenotypes observed in prion disease.
中文翻译:
ion病毒难以捉摸的原因。
仅蛋白质的假设认为,引起传染性海绵状脑病的传染原由蛋白质组成,缺乏任何信息性核酸。这种药物被斯坦利·普鲁西纳(Stanley Prusiner)称为病毒(prion),被认为部分由PrP(Sc)组成,PrP(Sc)是正常细胞蛋白的结构异构体,称为PrP(C)。and病毒概念使科学家和非专业人士着迷,并且它受到了热情的批评和严格的实验审查。结果,PrP(C)及其同工型位居哺乳动物基因组编码最深入研究的蛋白质之列。尽管进行了所有这些研究,但PrP(C)的生理功能和导致病毒疾病神经变性的分子途径仍然未知。
更新日期:2019-11-01
中文翻译:
ion病毒难以捉摸的原因。
仅蛋白质的假设认为,引起传染性海绵状脑病的传染原由蛋白质组成,缺乏任何信息性核酸。这种药物被斯坦利·普鲁西纳(Stanley Prusiner)称为病毒(prion),被认为部分由PrP(Sc)组成,PrP(Sc)是正常细胞蛋白的结构异构体,称为PrP(C)。and病毒概念使科学家和非专业人士着迷,并且它受到了热情的批评和严格的实验审查。结果,PrP(C)及其同工型位居哺乳动物基因组编码最深入研究的蛋白质之列。尽管进行了所有这些研究,但PrP(C)的生理功能和导致病毒疾病神经变性的分子途径仍然未知。