. Author manuscript; available in PMC: 2016 Feb 11.

Published in final edited form as: Front Biosci (Landmark Ed). 2016 Jan 1;21:42–54. doi: 10.2741/4375

Table 1.

Mitochondrial DNA repair enzymes

Enzyme	Class/Function
• *APE1*	*Incises at abasic sites*
• EXOG & DNA2	exonucleases that remove flaps
• *Pol γ*	*dRP lyase and gap filling*
• DUT1	hydrolyses dUTP to dUDP¹
• ENDOG	endonuclease²
• *Lig3*	*ligates repair patches or DS breaks*
• *MGMT: O6-methylguanine DNA methyltransferase*	*removes methyl group from O6-methylguanine*
• MTH1, MutT homolog	hydrolyzes 8-oxodGTP to 8-oxodGMP¹
• MYH, MutY homolog	removes A from 8-oxodG/A pairs
• *NEIL2, endonuclease VIII homolog*	*glycosylase/AP lyase removes oxidized bases*
• *NTH1, endonuclease III homolog*	*glycosylase/AP lyase removes oxidized pyrimidines*
• *OGG-1*	*glycosylase/AP lyase removes 8-oxo-guanine from oxo-G/C pairs*
• SSB	single-strand binding protein²
• TFAM	transcription factor A, member of the HMG family, binds DS DNA2
• *UNG, uracil gly*	*glycosylase, removes uracil*

removes damaged nucleotide triphosphate from dNTP pool protecting misincorporation during DNA replication

direct role in DNA repair has not been shown