Herzig Sébastien, Martinou Jean-Claude
Department of Cell Biology, University of Geneva, Quai Ernest-Ansermet 30, 1211 Geneva 4, Switzerland.
Curr Mol Med. 2008 Mar;8(2):131-7. doi: 10.2174/156652408783769625.
Mitochondria are essential organelles of all eukaryotic cells that play a key role in several physiological processes and are involved in the pathology of many diseases. These organelles form a highly dynamic network, which results from continuous fusion and fission processes. Importance of these processes is underlined by inherited human diseases caused by mutations in two mitochondrial pro-fusion genes: Charcot-Marie-Tooth disease, caused by mutations in Mitofusin 2 gene and ADOA due to mutations in OPA1. During apoptosis, the mitochondrial network is disintegrated and the outer mitochondrial membrane permeabilized, which results in the release of several apoptogenic proteins, including cytochrome c. Although modulating mitochondrial fusion and fission machineries has been reported to influence the apoptotic response to various stimuli, it is still unclear whether fission is absolutely required for apoptosis. In this review, we present the latest progress in the field of mitochondrial dynamics with a particular emphasis on its implication in apoptosis and in diseases.
线粒体是所有真核细胞中必不可少的细胞器,在多个生理过程中发挥关键作用,并与许多疾病的病理过程相关。这些细胞器形成一个高度动态的网络,这是由持续的融合和裂变过程导致的。两种线粒体促融合基因的突变所引起的人类遗传性疾病凸显了这些过程的重要性:由线粒体融合蛋白2基因突变导致的夏科-马里-图斯病以及由OPA1基因突变引起的常染色体显性视神经萎缩。在细胞凋亡过程中,线粒体网络解体,线粒体外膜通透性增加,这导致包括细胞色素c在内的几种凋亡蛋白的释放。尽管据报道调节线粒体融合和裂变机制会影响对各种刺激的凋亡反应,但目前仍不清楚裂变是否是细胞凋亡绝对必需的。在这篇综述中,我们介绍了线粒体动力学领域的最新进展,特别强调了其在细胞凋亡和疾病中的意义。