Santel Ansgar, Frank Stephan
Silence Therapeutics AG, Otto Warburg Haus (Nr. 80), Robert-Rössle-Strasse 10, D-13125 Berlin, Germany.
IUBMB Life. 2008 Jul;60(7):448-55. doi: 10.1002/iub.71.
Mitochondria are essential and dynamic cellular organelles differing in size, subcellular distribution, and internal structure. These aspects of mitochondrial morphology are intimately controlled by a growing number of mitochondrial morphology shaping proteins. The past decade has revealed remarkable and often unexpected new insights into the molecular regulation and physiological impact of mitochondrial morphology maintenance. Obviously, proper mitochondrial dynamics, resulting from a tightly regulated equilibrium between opposing mitochondrial fusion and fission activities, is a prerequisite for normal organelle function. Consequently, a disturbance of these activities results in mitochondrial dysfunction and, thus, can lay the foundation for human disorders. Here we specifically focus on recent advances in our understanding of the regulation, activity, and function of dynamin-related protein 1, the main factor for controlled mitochondrial fission.
线粒体是必不可少且具有动态变化的细胞器，其大小、亚细胞分布和内部结构各不相同。线粒体形态的这些方面受到越来越多的线粒体形态塑造蛋白的密切控制。在过去十年中，人们对线粒体形态维持的分子调控和生理影响有了显著且常常出乎意料的新见解。显然，由线粒体融合与裂变活动之间严格调控的平衡所产生的适当线粒体动力学，是正常细胞器功能的先决条件。因此，这些活动的紊乱会导致线粒体功能障碍，进而可能为人类疾病奠定基础。在这里，我们特别关注在理解动力相关蛋白1（线粒体可控裂变的主要因素）的调控、活性和功能方面的最新进展。
