线粒体蛋白质组及蛋白质量控制

Mitochondrial Proteome and Its Protein Quality Control

线粒体是哺乳动物细胞内重要细胞器,不仅通过氧化磷酸化产生ATP为细胞提供能量,也参与调节钙离子稳态、活性氧(reactive oxygen species,ROS)的产生、细胞应激反应和细胞死亡等过程,其功能障碍不仅导致多种人类疾病的发生,而且也能降低动物卵母细胞质量和早期胚胎发育能力。大量证据表明,线粒体的功能依赖于线粒体蛋白质组完整性和稳态。基于此,该文综述了线粒体蛋白组、线粒体蛋白转运,聚焦蛋白酶、分子伴侣、线粒体囊泡、线粒体自噬和线粒体未折叠蛋白反应在帮助正确的蛋白质折叠,去除错误折叠或聚集的蛋白质和清除功能失调的线粒体方面的作用,为调控线粒体蛋白质量,从而维持线粒体健康、降低疾病发生提供理论依据。

Mitochondria are one of the important intracellular organelles in mammals,which not only provide energy for cells by generating ATP via oxidative phosphorylation,but also participate in the regulation of calcium homeostasis,ROS(reactive oxygen species)production,cell stress response and cell death.Mitochondria dysfunction not only leads to a variety of human diseases,but also reduces the quality of animal oocytes and early embryonic development competence.Numerous studies show that the function of mitochondria depends on the integrity and homeostasis of mitochondrial proteome.Based on this,this paper reviewed the mitochondrial proteome,mitochondrial protein transport and the formation of functional protein network,and highlighted the role of proteases,molecular chaperones,mitochondrial-derived vesicles,mitophagy and mitochondrial unfolded protein response in helping correct protein folding,removing misfolded or aggregated proteins and eliminating dysfunctional mitochondria.This might provide reference for maintaining mitochondrial health and reducing the occurance of disease incidence via the modulation of mitochondrial protein quality.