线粒体是细胞内高度动态变化的细胞器,其在细胞内不断运动、融合、分裂并形成动态平衡的网状结构。线粒体的融合和分裂是由多种蛋白精确调控完成。Mfns/Fz01P控制线粒体外膜的融合,而Mgmlp/OPA1则参与线粒体内膜的融合;Dnm1p/Drp1、Fis1p/Fis1和Mdv1p介导线粒体分裂的调控。线粒体形态对于细胞维持正常生理代谢和机体发育起着重要的作用,一旦调控出现障碍会导致严重的疾病。
Mitochondria are highly dynamic organelles in a living cell, and display continuous movement, fusion and fission to form mitochondrial reticulum. Mitochondrial fusion and fission are regulated by a number of proteins. Mitochondrial fusion is mediated by the out-membrane protein, fuzzy onions (Fzolp) in yeast, or its homologues mitofusion 1 (Mfn1) and Mfn2 in mammalian. An intermembrane protein, Mgmlp in yeast and OPAl in mammalian, are involved in inner membrane fusion. On the other hand, mitochondrial fission is regulated by several proteins called Dnmlp, Fislp and Mdvlp in yeast and Drpl, Fisl in mammalian. The normal mitochondrial morphology is important for the function and physiology of mitochondria and cells, and defects in mitochondrial dynamics may cause severe diseases.