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| ABCB7 | COX15 | ISCU | NDUFA9 | NDUFS6 | SDHAF1 | UQCRB |
| ACAD9 | COX20 | LETM1 | NDUFAF1 | NDUFS7 | SDHB | UQCRC2 |
| APOPT1 | COX6A2 | LRPPRC | NDUFAF2 | NDUFS8 | SDHC | UQCRFS1 |
| ATP5F1A | COX6B1 | LYRM7 | NDUFAF3 | NDUFV1 | SDHD | UQCRQ |
| ATP5F1D | COX8A | MFN2 | NDUFAF4 | NDUFV2 | SPG7 | |
| ATP5MD | CYC1 | MRPS16 | NDUFAF5 | NFU1 | SURF1 | |
| ATPAF2 | DARS2 | MRPS2 | NDUFAF6 | NUBPL | TACO1 | |
| BCS1L | DNAJC19 | MRPS22 | NDUFAF8 | OPA1 | TAZ | |
| C19orf70 | DNM1L | MRPS34 | NDUFB11 | PDSS1 | TIMM8A | |
| C1QBP | FASTKD2 | NDUFA1 | NDUFB8 | PDSS2 | TIMMDC1 | |
| COA3 | FOXRED1 | NDUFA11 | NDUFB9 | PET100 | TMEM126B | |
| COQ2 | FXN | NDUFA12 | NDUFS1 | PUS1 | TRIT1 | |
| COQ8A | GFM1 | NDUFA2 | NDUFS2 | SCO1 | TSFM | |
| COX10 | GLRX5 | NDUFA6 | NDUFS3 | SCO2 | TTC19 | |
| COX14 | HSPD1 | NDUFA8 | NDUFS4 | SDHA | TUFM |
