哺乳动物线粒体DNA(mtDNA)通过噬菌体样DNA和RNA聚合酶进行了复制和转录,并且在过去的几十年中,我们对这些过程的理解取得了大幅度的发展。分子机制已通过生物化学和结构生物学阐明,并且由细胞生物学和小鼠遗传学确立的体内角色必不可少。Single molecules of mtDNA are packaged by mitochondrial transcription factor A into mitochondrial nucleoids, and their level of compaction influ- ences the initiation of both replication and transcription.Mutations affecting the molecular machineries replicating and transcribing mtDNA are impor- tant causes of human mitochondrial disease, reflecting the critical role of the genome in oxidative磷酸化系统生物发生。仍然需要澄清蒙德纳复制和转录的机制,并且该领域的未来研究可能会为治疗线粒体功能障碍的新型治疗可能性开放。
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