Abstract

This chapter describes the identification of lyase/dRpase activities in mitochondrial polymerase γ/and ligase; it also examined the substrates for mitochondrial (mt)DNA repair, as well as the cell specific differences and biological consequences of mtDNA repair; and Bohr examined oxidative DNA damage repair in mtDNA and the correlation of increased repair capacity with age. Mitochondrial DNA does not code for any DNA damage processing enzymes, but only for the oxidative phosphorylation proteins and some transfer (t)RNAs. Thus, all repair enzymes functioning in the mitochondria need to be transported in.

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