Number matters: control of mammalian mitochondrial DNA copy number

Laura L. Clay Montier, Janice J. Deng, Yidong Bai

Research output: Contribution to journalArticlepeer-review

391 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)125-131
Number of pages7
JournalJournal of Genetics and Genomics
Issue number3
StatePublished - Mar 2009


  • DNA copy number
  • DNA turnover
  • mitochondria
  • replication

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics


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