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Biochim Biophys Acta. 2012 Sep-Oct;1819(9-10):979-91. doi: 10.1016/j.bbagrm.2012.06.002. Epub 2012 Jun 19.

Mitochondrial DNA damage and its consequences for mitochondrial gene expression.

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1
Division of Basic Medical Sciences, Mercer University School of Medicine, Macon, GA 31207, USA. Cline_sd@mercer.edu

Abstract

How mitochondria process DNA damage and whether a change in the steady-state level of mitochondrial DNA damage (mtDNA) contributes to mitochondrial dysfunction are questions that fuel burgeoning areas of research into aging and disease pathogenesis. Over the past decade, researchers have identified and measured various forms of endogenous and environmental mtDNA damage and have elucidated mtDNA repair pathways. Interestingly, mitochondria do not appear to contain the full range of DNA repair mechanisms that operate in the nucleus, although mtDNA contains types of damage that are targets of each nuclear DNA repair pathway. The reduced repair capacity may, in part, explain the high mutation frequency of the mitochondrial chromosome. Since mtDNA replication is dependent on transcription, mtDNA damage may alter mitochondrial gene expression at three levels: by causing DNA polymerase γ nucleotide incorporation errors leading to mutations, by interfering with the priming of mtDNA replication by the mitochondrial RNA polymerase, or by inducing transcriptional mutagenesis or premature transcript termination. This review summarizes our current knowledge of mtDNA damage, its repair, and its effects on mtDNA integrity and gene expression. This article is part of a special issue entitled: Mitochondrial Gene Expression.

PMID:
22728831
PMCID:
PMC3412069
DOI:
10.1016/j.bbagrm.2012.06.002
[Indexed for MEDLINE]
Free PMC Article
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