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Mitochondrial DNA Polymerase Gamma (Pol γ)
- Target
- Pol γ
- Molecular classification
- Enzyme, DNA polymerase, Polymerase
01
Overview
Mitochondrial DNA polymerase gamma (Pol γ) is the sole DNA polymerase responsible for replication and repair of mitochondrial DNA (mtDNA) in animal cells. It is a heterotrimeric holoenzyme consisting of one catalytic subunit (Pol γA, encoded by POLG) and two accessory subunits (Pol γB, encoded by POLG2). Mutations in either the catalytic or accessory subunits can cause various mitochondrial disorders. Pol γ is also a target for some antiviral drugs, which can cause drug-induced mitochondrial toxicity.
- Other names
- POLGDNA polymerase subunit gammamtDNA polymerase gamma
02
Mechanism of action
Inhibition of mtDNA replication
03
Biological functions
Mitochondrial DNA replicationMitochondrial DNA repairDNA synthesisProofreadingBase excision repair
04
Disease associations
Mitochondrial disordersProgressive external ophthalmoplegiaAlpers syndromeAtaxia-neuropathy syndromes
05
Safety considerations
Drug-induced mitochondrial toxicityOff-target inhibition by antiviral drugs
06
Interacting drugs
AZT (zidovudine)
1 more in the full profile.
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