Disruption of RNA-binding proteins in neurological disorders

IF 4.6 2区 医学 Q1 NEUROSCIENCES Experimental Neurology Pub Date : 2024-12-19 DOI:10.1016/j.expneurol.2024.115119
Luyang Huang, Bo Zhao, Youzhong Wan
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Abstract

RNA-binding proteins (RBPs) are multifunctional proteins essential for the regulation of RNA processing and metabolism, contributing to the maintenance of cell homeostasis by modulating the expression of target genes. Many RBPs have been associated with neuron-specific processes vital for neuronal development and survival. RBP dysfunction may result in aberrations in RNA processing, which subsequently initiate a cascade of effects. Notably, RBPs are involved in the onset and progression of neurological disorders via diverse mechanisms. Disruption of RBPs not only affects RNA processing, but also promotes the abnormal aggregation of proteins into toxic inclusion bodies, and contributes to immune responses that drive the progression of neurological diseases. In this review, we summarize recent discoveries relating to the roles of RBPs in neurological diseases, discuss their contributions to such conditions, and highlight the unique functions of these RBPs within the nervous system.
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神经疾病中rna结合蛋白的破坏。
RNA结合蛋白(rbp)是调控RNA加工和代谢所必需的多功能蛋白,通过调节靶基因的表达来维持细胞稳态。许多rbp与对神经元发育和存活至关重要的神经元特异性过程有关。RBP功能障碍可能导致RNA加工畸变,随后引发一系列效应。值得注意的是,rbp通过多种机制参与神经系统疾病的发生和发展。rbp的破坏不仅影响RNA加工,还促进蛋白质异常聚集成毒性包涵体,并有助于驱动神经系统疾病进展的免疫反应。在这篇综述中,我们总结了与rbp在神经系统疾病中的作用有关的最新发现,讨论了它们对这些疾病的贡献,并强调了这些rbp在神经系统中的独特功能。
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来源期刊
Experimental Neurology
Experimental Neurology 医学-神经科学
CiteScore
10.10
自引率
3.80%
发文量
258
审稿时长
42 days
期刊介绍: Experimental Neurology, a Journal of Neuroscience Research, publishes original research in neuroscience with a particular emphasis on novel findings in neural development, regeneration, plasticity and transplantation. The journal has focused on research concerning basic mechanisms underlying neurological disorders.
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