RNA-mediated toxicity in neurodegenerative disease.
RNA-mediated toxicity in neurodegenerative disease.
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DOI：
10.1016/j.mcn.2012.12.006
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发表时间：
2013-09
影响因子：
3.5
通讯作者：
Petrucelli, Leonard
中科院分区：
文献类型：
--
作者：
Belzil, Veronique V.;Gendron, Tania F.;Petrucelli, Leonard
关键词：
细胞活力取决于由许多蛋白质编码和非编码RNA以及RNA结合蛋白执行的精心协调的功能。在过去的十年中，越来越明显的是，RNA加工异常是许多神经退行性疾病的共同特征。在包括由非编码重复扩增引起的疾病的“RNA病”中，RNA通过多种机制发挥毒性：RNA焦点形成、双向转录和通过重复相关的非ATG翻译产生毒性RNA和蛋白质。在“RNA结合蛋白病”中的毒性机制，其中RNA结合蛋白如TDP-43和FUS发挥突出作用的疾病，尚未完全阐明。尽管如此，RNA结合蛋白的功能丧失和由其聚集引起的功能的毒性获得两者被认为参与疾病发病机制。作为神经退行性变中RNA和剪接调节特刊的一部分，本文旨在探讨导致神经退行性变的各种RNA相关机制，特别强调动物模型中出现的发现。
Cellular viability depends upon the well-orchestrated functions carried out by numerous protein-coding and non-coding RNAs, as well as RNA-binding proteins. During the last decade, it has become increasingly evident that abnormalities in RNA processing represent a common feature among many neurodegenerative diseases. In “RNAopathies”, which include diseases caused by non-coding repeat expansions, RNAs exert toxicity via diverse mechanisms: RNA foci formation, bidirectional transcription, and the production of toxic RNAs and proteins by repeat associated non-ATG translation. The mechanisms of toxicity in “RNA-binding proteinopathies”, diseases in which RNA-binding proteins like TDP-43 and FUS play a prominent role, have yet to be fully elucidated. Nonetheless, both loss of function of the RNA binding protein, and a toxic gain of function resulting from its aggregation, are thought to be involved in disease pathogenesis. As part of the special issue on RNA and Splicing Regulation in Neurodegeneration, this review intends to explore the diverse RNA-related mechanisms contributing to neurodegeneration, with a special emphasis on findings emerging from animal models.
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