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DNA polymerase gamma (pol γ) is the primary and only known DNA polymerase responsible for replication and repair of mitochondrial DNA (mtDNA) in human cells. It is essential for maintaining mitochondrial genome integrity, which is critical for cellular energy production. The functional enzyme complex is a heterotrimer composed of one catalytic alpha subunit (POLG) and two accessory beta subunits (encoded by POLG2), forming a homodimer. Mutations or dysfunctions in POLG can lead to various mitochondrial diseases due to compromised mtDNA replication or increased mutation rates.
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