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泛素-蛋白酶体系统(UPS)是真核细胞内蛋白质降解的主要信号通路,UPS在降解细胞内错误折叠受损的蛋白也起着重要作用。神经退行性疾病的共同特征是中枢神经系统的细胞内或细胞外异常聚集着突变或损伤的蛋白,如帕金森病(PD)、阿尔茨海默病(AD)、亨廷顿病(Huntington)等。而且UPS成分的突变也可导致神经退行性疾病[1]。因此,UPS的功能异常影响着神经退行性疾病的发生发展。
Ubiquitin-proteasome system (UPS) is the major signal pathway for protein degradation in eukaryotes. UPS also plays an important role in misfolding damaged proteins in degraded cells. A common feature of neurodegenerative diseases is the abnormal or intracellular or extracellular aggregation of the central nervous system with mutated or impaired proteins such as Parkinson’s disease (PD), Alzheimer’s disease (AD), Huntington’s and others. And mutations in UPS components can also lead to neurodegenerative diseases [1]. Therefore, the abnormal function of UPS affects the development of neurodegenerative diseases.