Number matters： control of mammalian mitochondrial DNA copy number

• Published in: Journal of genetics and genomics Vol. 36; no. 3; pp. 125 - 131
• Main Authors: Clay Montier, Laura L., Deng, Janice J., Bai, Yidong
• Format: Journal Article
• Language: English
• Published: China Elsevier Ltd 01-03-2009
• Subjects: Animals; DNA; DNA copy number; DNA Replication; DNA turnover; DNA, Mitochondrial - genetics; DNA, Mitochondrial - metabolism; Gene Dosage; Humans; Mammals - genetics; Mammals - metabolism; mitochondria; Mitochondria - genetics; Mitochondria - metabolism; replication; 哺乳动物; 生源论; 遗传学; replication; DNA turnover; mitochondria; DNA copy number
• ISSN: 1673-8527
• DOI: 10.1016/s1673-8527(08)60099-5

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 ad- vanced 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.