| Gene Name | Chromosome number |
|---|---|
| FH | 1 |
| SDHC | 1 |
| SDHB | 1 |
| PARK7 | 1 |
| SUCLG1 | 2 |
| COX5B | 2 |
| PDK1 | 2 |
| PDHB | 3 |
| UQCRC1 | 3 |
| NNT | 5 |
| NDUFA2 | 5 |
| MDH2 | 7 |
| OGDH | 7 |
| PDK4 | 7 |
| CYCS | 7 |
| UQCRB | 8 |
| CYC1 | 8 |
| COX6C | 8 |
| LHPP | 10 |
| SDHD | 11 |
| LDHA | 11 |
| DLAT | 11 |
| PDHX | 11 |
| UCP2 | 11 |
| ATP5S | 14 |
| IDH2 | 15 |
| COX5A | 15 |
| ETFA | 15 |
| UQCRC2 | 16 |
| NDUFAB1 | 16 |
| PDK2 | 17 |
| COX11 | 17 |
| SCO1 | 17 |
| ATP5H | 17 |
| ETFB | 19 |
| BSG | 19 |
| ATP5E | 20 |
| ATP5J | 21 |
| ATP5O | 21 |
| UQCR10 | 22 |