Number matters: control of mammalian mitochondrial DNA copy number

被引:425
作者
Montier, Laura L. Clay [1 ]
Deng, Janice J. [1 ]
Bai, Yidong [1 ]
机构
[1] Univ Texas Hlth Sci Ctr San Antonio, Barshop Inst Longev & Aging Studies, Dept Cellular & Struct Biol, San Antonio, TX 78229 USA
关键词
mitochondria; DNA copy number; DNA turnover; replication; TRANSCRIPTION-FACTOR-A; GENE-EXPRESSION; POLYMERASE-GAMMA; ACCESSORY SUBUNIT; RAT HEPATOCYTES; MTDNA CONTENT; CELL-LINES; BIOGENESIS; REPLICATION; PROTEIN;
D O I
10.1016/S1673-8527(08)60099-5
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
引用
收藏
页码:125 / 131
页数:7
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