Article
Mutations in DNA polymerase gamma cause error prone DNA synthesis in human mitochondrial disorders.
Acta biochimica Polonica - 1 Jan 2003
Copeland William C, Ponamarev Mikhail V, Nguyen Dinh, Kunkel Thomas A, Longley Matthew J
Abstract excerpt
This paper summarizes recent advances in understanding the links between the cell's ability to maintain integrity of its mitochondrial genome and mitochondrial genetic diseases. Human mitochondrial DNA is replicated by the two-subunit DNA polymerase gamma (polgamma). We investigated the fidelity of DNA replication by polgamma with and without exonucleolytic proofreading and its p55 accessory subunit. Polgamma has...
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