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首页> 外文期刊>Drug news & perspectives >The role of ubiquitin C-terminal hydrolase L1 in neurodegenerative disorders.
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The role of ubiquitin C-terminal hydrolase L1 in neurodegenerative disorders.

机译：泛素C末端水解酶L1在神经退行性疾病中的作用。

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Impairment of the ubiquitin-proteasome system (UPS) results in the failure to remove and degrade misfolded proteins and consequently causes the accumulation of misfolded proteins in the cell. The aberrant interactions between misfolded proteins and normal intracellular proteins are thought to underlie the pathogenesis in many neurodegenerative diseases. Ubiquitin C-terminal hydrolase L1 (UCH-L1) is an important component of the UPS. Its major function is related to mono-ubiquitin recycling and thereby, sustaining protein degradation. Mutations of the UCH-L1 gene and alterations of its proteins' activity have been found to associate with several neurodegenerative disorders. In this review, we will discuss a link between UCH-L1 and Parkinson's, Huntington's and Alzheimer's diseases. We will also present a potential strategy for the treatment of Alzheimer's disease by boosting endogenous UCH-L1 activity.
机译：泛素-蛋白酶体系统（UPS）的损伤导致无法去除和降解错误折叠的蛋白质，从而导致错误折叠的蛋白质在细胞中积累。错误折叠的蛋白质和正常的细胞内蛋白质之间的异常相互作用被认为是许多神经退行性疾病发病机理的基础。泛素C末端水解酶L1（UCH-L1）是UPS的重要组成部分。它的主要功能与单遍在蛋白循环有关，从而维持蛋白质降解。已经发现UCH-L1基因的突变及其蛋白活性的改变与几种神经退行性疾病有关。在本文中，我们将讨论UCH-L1与帕金森氏症，亨廷顿氏病和阿尔茨海默氏病之间的联系。我们还将提出通过增强内源性UCH-L1活性来治疗阿尔茨海默氏病的潜在策略。

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