Role of the ubiquitin proteasome system in Parkinson's disease.
Role of the ubiquitin proteasome system in Parkinson's disease.
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DOI：
10.1186/1471-2091-8-s1-s13
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发表时间：
2007-11-22
期刊：
影响因子：
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通讯作者：
Tan, Jeanne M. M.
中科院分区：
文献类型：
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作者：
Lim, Kah-Leong;Tan, Jeanne M. M.
帕金森病（PD）是最常见的神经退行性运动障碍。虽然是一个深入研究的主题，但PD的病因仍然知之甚少。最近，一些证据表明泛素蛋白酶体系统（UPS）的畸变与PD发病机制之间存在密切联系。UPS通常作为一种蛋白质降解机制发挥作用，其失调会导致蛋白质稳态的改变，这可能会促进不利于神经元生存的蛋白质的毒性积累。毫不奇怪，基于UPS功能直接破坏的各种PD细胞和动物模型再现了PD的最突出特征。虽然有说服力，但过去几年的新发展实际上对UPS和PD之间的联系提出了严重的问题。在这里，我回顾目前的想法和争议，他们的关系，并讨论是否旨在减轻UPS功能障碍的策略可以代表合理的方式来干预疾病。出版历史：从Current BioData的靶向蛋白质数据库（TPdb;）重新出版。
Parkinson's disease (PD) is the most common neurodegenerative movement disorder. Although a subject of intense research, the etiology of PD remains poorly understood. Recently, several lines of evidence have implicated an intimate link between aberrations in the ubiquitin proteasome system (UPS) and PD pathogenesis. Derangements of the UPS, which normally functions as a type of protein degradation machinery, lead to alterations in protein homeostasis that could conceivably promote the toxic accumulation of proteins detrimental to neuronal survival. Not surprisingly, various cellular and animal models of PD that are based on direct disruption of UPS function reproduce the most prominent features of PD. Although persuasive, new developments in the past few years have in fact raised serious questions about the link between the UPS and PD. Here I review current thoughts and controversies about their relationship and discuss whether strategies aimed at mitigating UPS dysfunction could represent rational ways to intervene in the disease. Publication history: Republished from Current BioData's Targeted Proteins database (TPdb; ).