Zheng Qiuyang, Huang Timothy, Zhang Lishan, Zhou Ying, Luo Hong, Xu Huaxi, Wang Xin
Fujian Provincial Key Laboratory of Neurodegenerative Disease and Aging Research, Institute of Neuroscience, College of Medicine, Collaborative Innovation Center for Brain Science, Xiamen University Xiamen, China.
Neuroscience Initiative, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, CA USA.
Front Aging Neurosci. 2016 Dec 15;8:303. doi: 10.3389/fnagi.2016.00303. eCollection 2016.
The ubiquitin-proteasome system (UPS) is one of the major protein degradation pathways, where abnormal UPS function has been observed in cancer and neurological diseases. Many neurodegenerative diseases share a common pathological feature, namely intracellular ubiquitin-positive inclusions formed by aggregate-prone neurotoxic proteins. This suggests that dysfunction of the UPS in neurodegenerative diseases contributes to the accumulation of neurotoxic proteins and to instigate neurodegeneration. Here, we review recent findings describing various aspects of UPS dysregulation in neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, and Huntington's disease.
泛素-蛋白酶体系统（UPS）是主要的蛋白质降解途径之一，在癌症和神经疾病中已观察到UPS功能异常。许多神经退行性疾病具有一个共同的病理特征，即由易于聚集的神经毒性蛋白形成细胞内泛素阳性包涵体。这表明神经退行性疾病中UPS功能障碍导致神经毒性蛋白的积累并引发神经退行性变。在此，我们综述了描述诸如阿尔茨海默病、帕金森病和亨廷顿病等神经退行性疾病中UPS失调各方面的最新研究结果。
