TABLE 1.
Proteins associated with mitochondrial quality control.
| Mitochondrial quality control pathway | Protein | Molecular function |
| Mitochondrial proteostasis | HSP10/60/70/90 | Chaperones mediating protein folding |
| CLPP/LONP1/AFG3L2/SPG7/YME1L | Mitochondrial proteases mediating degradation | |
| ATFS1/ATF5/ATF4/FOXO3 | Transcription factors stimulating the UPRmt response | |
| UBL5/DVE1/CHOP/JNK/c-Jun | Co-transcription factors involved in the UPRmt response | |
| eIF2α/GCN2/PERK | Integrated stress response-related proteins | |
| PGC1-α/NRF | Transcription factors regulating mitochondrial biogenesis | |
| SIRT/SOD2 | Mitochondrial deacetylases and superoxide dismutase involved in antioxidant regulation and proteostasis | |
| Mitochondrial dynamics | OPA1 | IMM GTPase regulating mitochondrial fusion |
| MFN1/2 | OMM GTPases regulating mitochondrial fusion, transport, and mitophagy | |
| OMA1/YME1L1 | Mitochondrial inner membrane proteases | |
| DRP1 | Cytosolic GTPase regulating mitochondrial division | |
| MFF/Fis1/MiD49/51 | OMM adaptor proteins for DRP1 during mitochondrial division | |
| Kinesin/dynein | Motor proteins mediating mitochondrial axonal transport | |
| Miro/TRAK | Adaptor proteins connecting motor proteins to mitochondria | |
| Mitophagy | PINK1 | Kinase phosphorylating Parkin |
| Parkin | E3-ubiquitin ligase targeting OMM proteins | |
| VDAC/Mitofusin | OMM proteins | |
| MPP/PARL | Mitochondrial proteases cleaving PINK1 in healthy mitochondria | |
| OPTN/TAX1BP1/P62 | Autophagy receptor proteins | |
| VCP/FUNDC1/BNIP3 | Mitophagy receptor proteins | |
| ATG5/ATG7LC3 | Proteins mediating autophagy substrate selection and formation of autophagosomes | |
| UBXN1/UBXD1 | UBX domain-containing co-factors | |
| USP30 | Deubiquitinase localized on mitochondria | |
| GAPDH | Protein mediating glycolysis and serves as a mitophagy adaptor | |
| Others | SIRT3 | Mitochondrial deacetylase regulating antioxidants, proteostasis, and mitophagy |
| AMPK | Mitochondrial fuel sensor | |
| LRRK2 | PD-associated risk factor; kinase that phosphorylates DRP1 and Miro | |
| CHCHD10 | MICOS subunit regulating cristae structure and mitochondrial contact site | |
| TOM20 | Translocase of the OMM | |
| TBK1 | Kinase regulating apoptosis, autophagy, and inflammation | |
| PP1 | Protein phosphatase dephosphorylating DRP1 |