Chen Xin Jie, Butow Ronald A
Department of Molecular Biology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Dallas, Texas 75390-9148, USA.
Nat Rev Genet. 2005 Nov;6(11):815-25. doi: 10.1038/nrg1708.
Mitochondrial DNA (mtDNA) encodes essential components of the cellular energy-producing apparatus, and lesions in mtDNA and mitochondrial dysfunction contribute to numerous human diseases. Understanding mtDNA organization and inheritance is therefore an important goal. Recent studies have revealed that mitochondria use diverse metabolic enzymes to organize and protect mtDNA, drive the segregation of the organellar genome, and couple the inheritance of mtDNA with cellular metabolism. In addition, components of a membrane-associated mtDNA segregation apparatus that might link mtDNA transmission to mitochondrial movements are beginning to be identified. These findings provide new insights into the mechanisms of mtDNA maintenance and inheritance.
线粒体DNA（mtDNA）编码细胞能量产生装置的必需成分，mtDNA损伤和线粒体功能障碍会导致多种人类疾病。因此，了解mtDNA的组织和遗传是一个重要目标。最近的研究表明，线粒体利用多种代谢酶来组织和保护mtDNA，驱动细胞器基因组的分离，并将mtDNA的遗传与细胞代谢联系起来。此外，一种可能将mtDNA传递与线粒体运动联系起来的膜相关mtDNA分离装置的成分也开始被识别。这些发现为mtDNA维持和遗传机制提供了新的见解。
