泛素-蛋白酶体系统在家族性心肌病发生发展中的作用进展

Research progress of ubiquitin proteasome system in the development and progression of familial cardiomyopathy

泛素-蛋白酶体系统(UPS)可介导错误折叠和受损蛋白质的选择性降解,在许多信号通路中发挥着重要作用。肥厚型心肌病和扩张型心肌病是两种最常见的家族遗传性心肌病,主要为常染色体显性遗传,由编码心肌细胞成分的基因突变引起。本综述对遗传性心肌病中UPS损伤机制及治疗方案的研究进展进行了文献复习。

Ubiquitin-proteasome system(UPS),which mediates selective degradation of misfolding and damaged proteins,plays an important role in many signaling pathways.Hypertrophic cardiomyopathy and dilated cardiomyopathy are the two most common family inherited cardiomyopathies,mainly inherited in an autosomal dominant manner and are caused by mutations in genes that encode the components of cardiac muscle cells.We reviewed the literatures on the mechanism and treatment of UPS injury in hereditary cardiomyopathy.