Maria Falkenberg, Nils-Göran Larsson, Claes M. Gustafsson
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引用次数: 0
摘要
哺乳动物线粒体 DNA(mtDNA)通过噬菌体 DNA 和 RNA 聚合酶进行复制和转录,在过去的几十年中,我们对这些过程的了解取得了长足的进步。生物化学和结构生物学已经阐明了这些过程的分子机制,细胞生物学和小鼠遗传学也确定了这些过程在体内的重要作用。线粒体转录因子 A 将单分子 mtDNA 包装成线粒体核仁,其压实程度影响复制和转录的启动。影响复制和转录 mtDNA 分子机制的突变是导致人类线粒体疾病的重要原因,这反映了基因组在氧化磷酸化系统生物生成中的关键作用。控制 mtDNA 复制和转录的机制仍有待澄清,而这一领域的未来研究很可能为治疗线粒体功能障碍带来新的治疗可能性。
Replication and Transcription of Human Mitochondrial DNA
Mammalian mitochondrial DNA (mtDNA) is replicated and transcribed by phage-like DNA and RNA polymerases, and our understanding of these processes has progressed substantially over the last several decades. Molecular mechanisms have been elucidated by biochemistry and structural biology and essential in vivo roles established by cell biology and mouse genetics. Single molecules of mtDNA are packaged by mitochondrial transcription factor A into mitochondrial nucleoids, and their level of compaction influences the initiation of both replication and transcription. Mutations affecting the molecular machineries replicating and transcribing mtDNA are important causes of human mitochondrial disease, reflecting the critical role of the genome in oxidative phosphorylation system biogenesis. Mechanisms controlling mtDNA replication and transcription still need to be clarified, and future research in this area is likely to open novel therapeutic possibilities for treating mitochondrial dysfunction.
期刊介绍:
The Annual Review of Biochemistry, in publication since 1932, sets the standard for review articles in biological chemistry and molecular biology. Since its inception, these volumes have served as an indispensable resource for both the practicing biochemist and students of biochemistry.