| 2-Oxoadipate dehydrogenase complex | OADC | | |
| 2-Oxoglutarate dehydrogenase complex | OGDC | | |
| 3-Methylcrotonyl-CoA carboxylase | MCCC | | |
| ATP synthase | RC5 | | |
| Branched-chain 2-oxoacid dehydrogenase complex | BCKDC | | |
| DNA polymerase gamma | POLG | | |
| Electron transfer flavoprotein | ETF | | |
| Glycine cleavage system | GCS | | |
| Hepatic phosphorylase kinase | hPhK | | |
| Isocitrate dehydrogenase 3 | IDH3 | | |
| Lactate dehydrogenase | LDH | | |
| Large neutral amino acid transporter | LAT | | |
| Mitochondrial ATP-dependent CLP protease | mClpP | | |
| Mitochondrial glutamyl-tRNA amidotransferase | mGAT | | |
| Mitochondrial isocitrate dehydrogenase | mIDH | | |
| Mitochondrial ribonucleotide reductase | RRM | | |
| Mitochondrial ribosomal large subunit | mRPL | | |
| Mitochondrial ribosomal small subunit | mRPS | | |
| Mitochondrial trifunctional protein | MTP | | |
| Mitochondrial-processing peptidase | PMP | | |