Gao Xuechao, Hu Hongyu
State Key Laboratory of Molecular Biology, Institute of Biochemistry and Cell Biology, Shanghai Institutes for Biological Sciences, Chinese Academy of Sciences, Shanghai 200031, China.
Acta Biochim Biophys Sin (Shanghai). 2008 Jul;40(7):612-8. doi: 10.1111/j.1745-7270.2008.00441.x.
Most neurodegenerative diseases including Alzheimer's disease, Parkinson's disease, Huntington's disease and other polyglutamine diseases are associated with degeneration and death of specific neuronal populations due to misfolding or aggregation of certain proteins. These aggregates often contain ubiquitin that is the signal for proteolysis by the ubiquitin-proteasome system, and chaperone proteins that are involved in the assistance of protein folding. Here we review the role of protein quality control systems in the pathogenesis of neurodegenerative diseases, and aim to learn more from the cooperation between molecular chaperones and ubiquitin-proteasome system responding to cellular protein aggregates, in order to find molecular targets for therapeutic intervention.
大多数神经退行性疾病，包括阿尔茨海默病、帕金森病、亨廷顿病和其他多聚谷氨酰胺疾病，都与特定神经元群体的退化和死亡有关，这是由某些蛋白质的错误折叠或聚集所导致的。这些聚集体通常含有泛素，它是泛素-蛋白酶体系统进行蛋白水解的信号，还含有参与协助蛋白质折叠的伴侣蛋白。在此，我们综述蛋白质质量控制系统在神经退行性疾病发病机制中的作用，旨在从分子伴侣与泛素-蛋白酶体系统针对细胞内蛋白质聚集体的协同作用中获得更多认识，以便找到治疗干预的分子靶点。
