Table 1.
Tissue specificity, cellular localization, and known or inferred functions of mitochondrial isoforms of selected BER glycosylases and MTH1.
| Enzyme | Glycosylase Family | Tissue Specificity | Inferred or Confirmed Function(s) of Mitochondrial Isoform | Mitochondrial Localization Described |
|---|---|---|---|---|
| NEIL1 | Fpg/Nei Helix-two turns-helix | Liver, thymus, pancreas, brain [128,129,130] | Potential role in mediating metabolic syndrome in Neil1−/− mice [141]; Binding partner for mtSSB [142] | [142,143] |
| NEIL2 | Fpg/Nei Helix-two turns-helix | Testes and skeletal muscle [131] | Removal of oxidized bases from mitochondrial genome [144] | [144] |
| OGG1 (1a) | Helix-hairpin- helix | Thymus, testis, intestine, brain, and germinal center of B cells [130,133,135,145] | Role in protecting against oxidative stress [146,147,148,149]; Prevention of metabolic syndrome [116] | [150,151,152] |
| NTH1 | Helix-hairpin- helix | Heart, brain [130,132,153] | Unknown; potentially compensated for by NEIL1 activity | Mouse isoform is exclusively mitochondrial; human isoform is thought to be exclusively nuclear [154,155] |
| MUTY | Helix-hairpin- helix | Thymus, intestine, heart, lung [156] | Potentially involved in repair of hypoxia induced damage in brain [157] | [156,158] |
| MTH1 | Oxidized purine nucleoside triphosphatase | Thymus, testis, embryonic tissues [127] | Protection from oxidative damage in models of Parkinson’s disease [137] | [136,138,159] |