Effect of host and strain factors on α-synuclein prion pathogenesis.

IF 14.6 1区 医学 Q1 NEUROSCIENCES
Trends in Neurosciences Pub Date : 2024-07-01 Epub Date: 2024-05-27 DOI:10.1016/j.tins.2024.05.004
Amanda L Woerman, Jason C Bartz
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引用次数: 0

Abstract

Prion diseases are a group of neurodegenerative disorders caused by misfolding of proteins into pathogenic conformations that self-template to spread disease. Although this mechanism is largely associated with the prion protein (PrP) in classical prion diseases, a growing literature indicates that other proteins, including α-synuclein, rely on a similar disease mechanism. Notably, α-synuclein misfolds into distinct conformations, or strains, that cause discrete clinical disorders including multiple system atrophy (MSA) and Parkinson's disease (PD). Because the recognized similarities between PrP and α-synuclein are increasing, this review article draws from research on PrP to identify the host and strain factors that impact disease pathogenesis, predominantly in rodent models, and focuses on key considerations for future research on α-synuclein prions.

宿主和菌株因素对α-突触核蛋白朊病毒发病机制的影响
朊病毒病是一组神经退行性疾病,由蛋白质错误折叠成致病构象引起,这种构象会自我模板化以传播疾病。尽管这种机制主要与经典朊病毒疾病中的朊病毒蛋白(PrP)有关,但越来越多的文献表明,包括α-突触核蛋白在内的其他蛋白质也依赖于类似的疾病机制。值得注意的是,α-突触核蛋白会错误地折叠成不同的构象或品系,从而导致不同的临床疾病,包括多系统萎缩症(MSA)和帕金森病(PD)。由于公认的 PrP 和 α-synuclein 之间的相似性越来越多,这篇综述文章借鉴了 PrP 的研究,主要在啮齿类动物模型中确定了影响疾病发病机制的宿主和菌株因素,并重点介绍了未来研究 α-synuclein 朊病毒的主要考虑因素。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Trends in Neurosciences
Trends in Neurosciences 医学-神经科学
CiteScore
26.50
自引率
1.30%
发文量
123
审稿时长
6-12 weeks
期刊介绍: For over four decades, Trends in Neurosciences (TINS) has been a prominent source of inspiring reviews and commentaries across all disciplines of neuroscience. TINS is a monthly, peer-reviewed journal, and its articles are curated by the Editor and authored by leading researchers in their respective fields. The journal communicates exciting advances in brain research, serves as a voice for the global neuroscience community, and highlights the contribution of neuroscientific research to medicine and society.
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