title: The role of mitochondrial DNA mutations in mammalian aging.
abstract: Mitochondrial DNA (mtDNA) accumulates both base-substitution mutations and deletions with aging in several tissues in mammals. Here, we examine the evidence supporting a causative role for mtDNA mutations in mammalian aging. We describe and compare human diseases and mouse models associated with mitochondrial genome instability. We also discuss potential mechanisms for the generation of these mutations and the means by which they may mediate their pathological consequences. Strategies for slowing the accumulation and attenuating the effects of mtDNA mutations are discussed.
authors.affiliation: Department of Genetics and Medical Genetics, University of Wisconsin, Madison, Wisconsin, United States of America.
authors.forename: Gregory C
authors.initials: GC
authors.lastname: Kujoth
authors.forename: Patrick C
authors.initials: PC
authors.lastname: Bradshaw
authors.forename: Suraiya
authors.initials: S
authors.lastname: Haroon
authors.forename: Tomas A
authors.initials: TA
authors.lastname: Prolla
mesh_terms: D000375:Aging / Q000235:genetics*; D000818:Animals; D004272:DNA, Mitochondrial / Q000235:genetics*; D004195:Disease Models, Animal; D030342:Genetic Diseases, Inborn / Q000209:etiology; D042822:Genomic Instability; D006801:Humans; D051379:Mice; D009154:Mutation / Q000502:physiology*
chemical_list: D004272:DNA, Mitochondrial