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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。
Mitochondria are dynamic cellular organelles with a bilayer membrane structure that is an important site for energy synthesis in eukaryotic cells. Mitochondrial inner membrane to the mitochondrial matrix protruding mitochondrial crista, mitochondrial cristae mitochondria in the orderly arrangement of the mitochondrial ATP production is an important place, but about the formation of cristae and biological function of little. In recent years, the newly discovered MICOS complex is considered as a key complex regulating mitochondrial crista morphology, and MICOS complexes are found to exert a variety of mitochondrial-related biological functions in cells. This article focuses on the various subunit proteins and properties of MICOS complexes and the important functions of MICOS complexes and their relationship to major human diseases to promote understanding and exploration of MICOS complexes.