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The accuracy of mitochondrial DNA (mtDNA) replication depends on the coordinated action of many nuclear-encoded proteins and on the correct balance of nucleotides within the mitochondrial matrix. mtDNA is replicated by DNA polymerase gamma, which is composed of a 140-kD catalytic subunit (POLG1; MIM 174763) and a 55-kD accessory subunit (POLG2).[supplied by OMIM]
