MICOS复合物/线粒体内膜结构调控关键复合物的研究进展

MICOS Complex Is Essential for Regulation of Mitochondrial Membrane Organization

线粒体是具有双层膜结构的动态细胞器,它是真核生物细胞内能量合成的重要场所。线粒体内膜向线粒体基质处突起形成线粒体嵴,线粒体嵴在线粒体内整齐有序地排列,是线粒体内产生ATP的重要场所,但关于嵴的形成机制和生物学功能知之甚少。近年来新发现的MICOS复合物被认为是调控线粒体嵴形态的关键复合物,并且发现MICOS复合物在细胞中发挥多种线粒体相关的生物学功能。该文重点介绍MICOS复合物的各个亚基蛋白和特性以及MICOS复合物的重要功能及其与人类重大疾病的关系,以促进对于MICOS复合物的认识及探索。

Mitochondrion is a dynamic double-membrane-bounded organelle; it is the main site of energy conversion in eukaryotic cell. Mitochondrial cristae are subcompartments of mitochondrial inner membrane. They are arranged orderly and are main sites of ATP production in mitochondria, but the mechanism of cristae forma- tion is poorly known. Recently, MICOS complex was identified to be a key regulator in the cristae formation andmorphology, and it played a serious of biological functios in cells. This review will focus on the subunits of MICOS complex, introducing the important functions of MICOS complex and its relationship with human diseases, and giving an overlook of understanding in MICOS complex.