Number matters:control of mammalian mitochondrial DNA copy number

作     者：Laura L.Clay Montier Janice J.Deng 

作者机构：Department of Cellular and Structural BiologyThe Barshop Institute for Longevity and Aging Studies University of Texas Health Science Center at San Antonio 

出 版 物：《Journal of Genetics and Genomics》 (遗传学报（英文版）)

年 卷 期：2009年第36卷第3期

页      面：125-131页

核心收录：

学科分类：0710[理学-生物学] 1001[医学-基础医学(可授医学、理学学位)] 07[理学] 0905[农学-畜牧学] 09[农学] 071007[理学-遗传学] 090501[农学-动物遗传育种与繁殖] 

基　　金：supported by the National Institute of Aging/National Institution of Health  USA (No. AG025223 and AG024640) to YB 

主　　题：mitochondria DNA copy number DNA turnover replication 

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 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.