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神经疾病中RNA结合蛋白的破坏。

Disruption of RNA-binding proteins in neurological disorders.

作者信息

Huang Luyang, Zhao Bo, Wan Youzhong

机构信息

Cancer Biology Laboratory, China-Japan Union Hospital of Jilin University, Changchun 130062, Jilin, China.

Cancer Biology Laboratory, China-Japan Union Hospital of Jilin University, Changchun 130062, Jilin, China.

出版信息

Exp Neurol. 2025 Mar;385:115119. doi: 10.1016/j.expneurol.2024.115119. Epub 2024 Dec 19.

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.

摘要

RNA结合蛋白(RBPs)是多功能蛋白,对RNA加工和代谢的调控至关重要,通过调节靶基因的表达有助于维持细胞内稳态。许多RBPs与对神经元发育和存活至关重要的神经元特异性过程相关。RBP功能障碍可能导致RNA加工异常,进而引发一系列效应。值得注意的是,RBPs通过多种机制参与神经疾病的发生和发展。RBPs的破坏不仅影响RNA加工,还会促进蛋白质异常聚集形成有毒包涵体,并导致推动神经疾病进展的免疫反应。在本综述中,我们总结了关于RBPs在神经疾病中作用的最新发现,讨论它们对这些病症的贡献,并强调这些RBPs在神经系统中的独特功能。

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