| Autosomal recessive | Most common (e.g., SURF1, PDHA1, NDUFS1) |
| Maternal inheritance | MT-ATP6, MT-ND genes (mitochondrial DNA) |
| X-linked | PDHA1 (most common X-linked form) |
| Complex I genes | NDUFS1, NDUFS2, NDUFS4, NDUFAF6 |
| Complex IV genes | SURF1, COX10, COX15, SCO1 |
| Complex V genes | ATP5F1A (MTATP1) |
| Pyruvate dehydrogenase genes | PDHA1, PDHB, DLAT |
| Coenzyme Q genes | COQ8A (ADCK3), COQ9 |
| Assembly factors | Various complex-specific factors |
| MT-ATP6 | ATP synthase subunit 6 |
| MT-ND1, MT-ND5, MT-ND6 | Complex I subunits |
| MT-CO1, MT-CO2, MT-CO3 | Complex IV subunits |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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