ABSTRACT

Mitochondrial DNA (mtDNA) is a tiny double-stranded circular DNA located within the mitochondria of most tissues in mammals. Numerous copies of mtDNA exist in a single cell and its replication occurs continuously, even in cells that do not divide. Another key feature of mtDNA is its high sensitivity to oxidative damage, in comparison to the nuclear genome. Notably, this genome encodes for 13 polypeptides of the respiratory chain, which is mandatory for ATP synthesis and the oxidation of the

Abstract .................................................................................................................. 283 Mitochondrial Genetics, Bioenergetics, and Diseases ...........................................284