Mitochondrial DNA maintenance and bioenergetics.
Oxidative phosphorylation requires assembly of the protein products of both mitochondrial and of nuclear genomes into functional respiratory complexes. Cellular respiration can be compromised when mitochondrial DNA (mtDNA) sequences are corrupted. Oxidative damage resulting from reactive oxygen species (ROS) produced during respiration is probably a major source of mitochondrial genomic instability leading to respiratory dysfunction. Here, we review mechanisms of mitochondrial ROS production, mtDNA damage and its relationship to mitochondrial dysfunction. We focus particular attention on the roles of mtDNA repair enzymes and processes by which the integrity of the mitochondrial genome is maintained and dysfunction prevented.
Stuart
Jeffrey A
JA
Department of Biological Sciences, Brock University, St. Catharines, Ontario, Canada L2S 3A1. jstuart@brocku.ca
Brown
Melanie F
MF
D000818:Animals; D004249:DNA Damage / Q000502:physiology; D004260:DNA Repair / Q000502:physiology; D004272:DNA, Mitochondrial / Q000235:genetics*; D004734:Energy Metabolism / Q000502:physiology*; D006801:Humans; D008928:Mitochondria / Q000187:drug effects / Q000235:genetics / Q000378:metabolism*; D017382:Reactive Oxygen Species / Q000378:metabolism* / Q000494:pharmacology
D004272:DNA, Mitochondrial; D017382:Reactive Oxygen Species