Number matters: control of mammalian mitochondrial DNA copy number.
Number matters: control of mammalian mitochondrial DNA copy number.
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DOI：
10.1016/s1673-8527(08)60099-5
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发表时间：
2009-03
期刊：
影响因子：
--
通讯作者：
Bai Y
中科院分区：
文献类型：
--
作者：
Clay Montier LL;Deng JJ;Bai Y
线粒体生物发生的调节对于细胞的正常功能是必不可少的。线粒体DNA(MtDNA)耗竭和由此导致的线粒体功能障碍与癌症、神经退行性变、糖尿病、衰老和许多其他人类疾病有关。虽然已知哺乳动物线粒体基因组的动态与核基因组的动态没有关联，但对mtDNA的传播机制知之甚少。然而，我们对线粒体DNA复制模式的理解在最近几年有所进步，尽管也存在一些争议。本文综述了我们目前对哺乳动物细胞线粒体DNA拷贝数控制的认识，重点介绍了线粒体DNA的复制和翻转。尽管mtDNA拷贝数似乎过多，但我们认为mtDNA拷贝数控制是线粒体遗传学和生物发生的一个重要方面，对于正常的细胞功能是必不可少的。
Regulation of mitochondrial biogenesis is essential for proper cellular functioning. Mitochondrial DNA (mtDNA) depletion and the resulting mitochondrial malfunction have been implicated in cancer, neurodegeneration, diabetes, aging, and many other human diseases. Although it is known that the dynamics of the mammalian mitochondrial genome are not linked with that of the nuclear genome, very little is known about the mechanism of mtDNA propagation. Nevertheless, our understanding of the mode of mtDNA replication has advanced in recent years, though not without some controversies. This review summarizes our current knowledge of mtDNA copy number control in mammalian cells, while focusing on both mtDNA replication and turnover. Although mtDNA copy number is seemingly in excess, we reason that mtDNA copy number control is an important aspect of mitochondrial genetics and biogenesis and is essential for normal cellular function.