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Prion protein and prion disease at a glance.
Journal of Cell Science ( IF 4 ) Pub Date : 2021-09-02 , DOI: 10.1242/jcs.245605
Caihong Zhu 1 , Adriano Aguzzi 2
Affiliation  

Prion diseases are neurodegenerative disorders caused by conformational conversion of the cellular prion protein (PrPC) into scrapie prion protein (PrPSc). As the main component of prion, PrPSc acts as an infectious template that recruits and converts normal cellular PrPC into its pathogenic, misfolded isoform. Intriguingly, the phenomenon of prionoid, or prion-like, spread has also been observed in many other disease-associated proteins, such as amyloid β (Aβ), tau and α-synuclein. This Cell Science at a Glance and the accompanying poster highlight recently described physiological roles of prion protein and the advanced understanding of pathogenesis of prion disease they have afforded. Importantly, prion protein may also be involved in the pathogenesis of other neurodegenerative disorders such as Alzheimer's and Parkinson's disease. Therapeutic studies of prion disease have also exploited novel strategies to combat these devastating diseases. Future studies on prion protein and prion disease will deepen our understanding of the pathogenesis of a broad spectrum of neurodegenerative conditions.

中文翻译:

朊病毒蛋白和朊病毒病一目了然。

朊病毒病是由细胞朊病毒蛋白 (PrPC) 构象转化为痒病朊病毒蛋白 (PrPSc) 引起的神经退行性疾病。作为朊病毒的主要成分,PrPSc 充当感染性模板,可招募正常细胞 PrPC 并将其转化为其致病性错误折叠亚型。有趣的是,在许多其他与疾病相关的蛋白质中也观察到了朊病毒或朊病毒样传播现象,例如淀粉样蛋白 β (Aβ)、tau 和 α-突触核蛋白。这份细胞科学概览和随附的海报重点介绍了朊病毒蛋白的生理作用以及它们提供的对朊病毒病发病机制的深入理解。重要的是,朊病毒蛋白也可能参与其他神经退行性疾病的发病机制,如阿尔茨海默病和帕金森病。朊病毒病的治疗研究也利用了新的策略来对抗这些破坏性疾病。未来对朊病毒蛋白和朊病毒病的研究将加深我们对广谱神经退行性疾病发病机制的理解。
更新日期:2021-09-02
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