线粒体融合、分裂及其相关疾病的研究进展

近年来，有关线粒体融合和分裂机制的相关研究越来越多。线粒体是所有真核生物都不可或缺的双层膜细胞器，大多数细胞中线粒体是高度动态的，且通过不断地融合、分裂来维持动态平衡。线粒体外膜与内膜的融合分别由Mfn1、Mfn2与多种形式的OPA1调控；DRP1介导外膜分裂，内膜的分裂可能是由S-OPA1和MTP18介导。多种客观因素通过影响融合或分裂的程度，进而影响线粒体的融合与分裂，提高线粒体融合程度或降低线粒体分裂程度都将导致在细胞内形成个体大、数量少的线粒体；反之，将出现个体小、数量多的线粒体。可以据此特性间接检测某项影响线粒体形态学变化的因素，了解线粒体动态变化所涉及的蛋白，以及影响线粒体形态学的重要外部因素、线粒体相关疾病的发病原因。因此，作者在总结前人研究成果的基础上，针对线粒体融合与分裂、参与线粒体融合与分裂的相关蛋白及线粒体融合、分裂与疾病的发生进行了综述。

Research Progress on the Mitochondrial Fusion,Fission and Their Effects on Disease

In recent years, more and more studies on mitochondrial fusion and fragmentation mechanisms have been conducted, mitochondria are double-membrane organelles possessed by all eukaryotic organisms. In most cells, mitochondria are highly dynamic and maintain their homeostasis by continually fusing and dividing.Mitochondrial outer membrane and endometrial fusion were regulated, respectively by Mfn1, Mfn2 and a variety of forms of OPA1;DRP1 mediated epineurial division,at present the mechanism of endometrial fission is still unclear,may be mediated by S-OPA1 and MTP18. Research shows that a variety of objective factors through the impact of the degree of integration or division,thus affecting the mitochondrial fusion and fission,increased mitochondrial fusion or decreased mitochondrial fission will result in the cell into individual large, small number of mitochondria;On the contrary,will lead to mitochondria appear individual small, large number. In the future research process, this feature can be used to detect indirectly a change in mitochondrial morphology factors. On the basis of summarizing the results of previous studies, in the present review, we focus on the mitochondrial fusion and fission, some related proteins involved in mitochondrial fusion and fission, as well as the occurrence of mitochondrial disease.