RNA metabolism in neurodegenerative disease.
RNA metabolism in neurodegenerative disease.
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DOI：
10.1242/dmm.028613
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发表时间：
2017-05-01
影响因子：
4.3
通讯作者：
Lee EB
中科院分区：
文献类型：
--
作者：
Liu EY;Cali CP;Lee EB
衰老相关的神经退行性疾病是一种进行性和致命性的神经系统疾病，其特征是不可逆的神经元丢失和胶质细胞增生。随着老龄化人口的不断增加，迫切需要更好地了解这些疾病背后的基本生物学。尽管不同的疾病机制与神经退行性疾病有关，但RNA加工改变的一个共同主题已经成为神经退行性疾病的统一贡献因素。RNA加工包括一系列不同的过程，包括RNA剪接、运输和稳定，以及非编码RNA的生物发生。在这里，我们重点介绍其中一些机制是如何在神经退行性疾病中改变的，包括由微卫星重复、microRNA生物发生改变和tRNA生物发生缺陷引起的RNA结合蛋白的错误定位和隔离，以及长基因间非编码RNA的变化。我们还强调了针对每种机制的潜在治疗干预措施。摘要：在这篇综述中，Edward Lee和合著者概述了导致神经退行性疾病病理的RNA代谢缺陷，包括RNA结合蛋白的错误定位和微小RNA的生物发生改变。
Aging-related neurodegenerative diseases are progressive and fatal neurological diseases that are characterized by irreversible neuron loss and gliosis. With a growing population of aging individuals, there is a pressing need to better understand the basic biology underlying these diseases. Although diverse disease mechanisms have been implicated in neurodegeneration, a common theme of altered RNA processing has emerged as a unifying contributing factor to neurodegenerative disease. RNA processing includes a series of distinct processes, including RNA splicing, transport and stability, as well as the biogenesis of non-coding RNAs. Here, we highlight how some of these mechanisms are altered in neurodegenerative disease, including the mislocalization of RNA-binding proteins and their sequestration induced by microsatellite repeats, microRNA biogenesis alterations and defective tRNA biogenesis, as well as changes to long-intergenic non-coding RNAs. We also highlight potential therapeutic interventions for each of these mechanisms. Summary: In this At a Glance review, Edward Lee and co-authors provide an overview of RNA metabolism defects, including mislocalization of RNA-binding proteins and microRNA biogenesis alterations, that contribute to neurodegenerative disease pathology.