酵母线粒体ATP合酶生物合成及组装机制研究进展

线粒体ATP合酶是线粒体氧化磷酸化的关键酶,其功能缺陷会导致能量代谢障碍相关的线粒体疾病。线粒体ATP合酶是由多个亚基组成的蛋白复合物,其生物合成和组装是个复杂的生物过程。酵母是研究线粒体ATP合酶结构、生物合成和组装机制的模式实验材料之一,且相关研究取得了很多进展。本文概述了国内外用酿酒酵母研究线粒体ATP合酶的结构、调控线粒体ATP合酶亚基生物合成和组装的辅助蛋白及合酶的模块化组装过程的研究进展,以期为线粒体ATP合酶的工作机制及相关线粒体疾病的研究提供理论借鉴和参考依据。

Advance of researches on biogenesis and assembly of yeast mitochondrial ATP synthase

Mitochondrial ATP synthase is one of key enzymes of mitochondrial oxidative phosphorylation. Functional deficiency in mitochondrial ATP synthase can lead to energy metabolism related mitochondrial disorders. Mitochondrial ATP synthase consists of multiple subunits, and its biogenesis and assembly is a complicated biological process. Yeast is one of model organisms for studying structure, biogenesis and assembly mechanism of mitochondrial ATP synthase, and many progress has been made in related research. This review summarizes the research progress in the structure, biogenesis and assembly regulator factors, and modular assembly process of Saccharomyces cerevisiae mitochondrial ATP synthase, providing references for study of working mechanism of mitochondrial ATP synthase and of related mitochondrial diseases.