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| UQCRC1 | Cytochrome b-c1 complex subunit 1, mitochondrial; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This protein may mediate formation of the complex between cytochromes c and c1; M16 metallopeptidases (480 aa) | |||
| FA2H | Fatty acid 2-hydroxylase; Required for alpha-hydroxylation of free fatty acids and the formation of alpha-hydroxylated sphingolipids; Belongs to the sterol desaturase family. SCS7 subfamily (372 aa) | |||
| DNAJC17 | DnaJ homolog subfamily C member 17; May negatively affect PAX8-induced thyroglobulin/TG transcription; DNAJ heat shock proteins (304 aa) | |||
| DNAJC12 | DnaJ heat shock protein family member C12 (198 aa) | |||
| PMPCB | Mitochondrial-processing peptidase subunit beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors; M16 metallopeptidases (489 aa) | |||
| UQCRC2 | Cytochrome b-c1 complex subunit 2, mitochondrial; This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. The core protein 2 is required for the assembly of the complex; M16 metallopeptidases (453 aa) | |||
| TIMM44 | Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. Recruits mitochondrial HSP70 to drive protein translocation into the matrix using ATP as an energy source; Belongs to the Tim44 family (452 aa) | |||
| CYB5B | Cytochrome b5 type B; Cytochrome b5 is a membrane bound hemoprotein which function as an electron carrier for several membrane bound oxygenases (150 aa) | |||
| TIMM22 | Mitochondrial import inner membrane translocase subunit Tim22; Essential core component of the TIM22 complex, a complex that mediates the import and insertion of multi-pass transmembrane proteins into the mitochondrial inner membrane. In the TIM22 complex, it constitutes the voltage-activated and signal-gated channel. Forms a twin-pore translocase that uses the membrane potential as external driving force in 2 voltage-dependent steps (By similarity); Belongs to the Tim17/Tim22/Tim23 family (194 aa) | |||
| CYB5D1 | Cytochrome b5 domain containing 1 (228 aa) | |||
| CYB5A | Cytochrome b5; Cytochrome b5 is a membrane bound hemoprotein which function as an electron carrier for several membrane bound oxygenases (134 aa) | |||
| TIMM17A | Mitochondrial import inner membrane translocase subunit Tim17-A; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane (171 aa) | |||
| CYB5R4 | Cytochrome b5 reductase 4; NADH-cytochrome b5 reductase involved in endoplasmic reticulum stress response pathway. Plays a critical role in protecting pancreatic beta-cells against oxidant stress, possibly by protecting the cell from excess buildup of reactive oxygen species (ROS). Reduces a variety of substrates in vitro, such as cytochrome c, feericyanide and methemoglobin (521 aa) | |||
| PMPCA | Mitochondrial-processing peptidase subunit alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors; Belongs to the peptidase M16 family (525 aa) | |||
| DNAJC9 | DnaJ homolog subfamily C member 9; May play a role as co-chaperone of the Hsp70 family proteins HSPA1A, HSPA1B and HSPA8; DNAJ heat shock proteins (260 aa) | |||
| TIMM23B | Translocase of inner mitochondrial membrane 23 homolog B; TIM23 complex (188 aa) | |||
| HAO1 | Hydroxyacid oxidase 1; Has 2-hydroxyacid oxidase activity. Most active on the 2-carbon substrate glycolate, but is also active on 2-hydroxy fatty acids, with high activity towards 2-hydroxy palmitate and 2- hydroxy octanoate (370 aa) | |||
| DNAJC15 | DnaJ homolog subfamily C member 15; Negative regulator of the mitochondrial respiratory chain. Prevents mitochondrial hyperpolarization state and restricts mitochondrial generation of ATP (By similarity). Acts as an import component of the TIM23 translocase complex. Stimulates the ATPase activity of HSPA9; DNAJ heat shock proteins (150 aa) | |||
| DNAJC19 | Mitochondrial import inner membrane translocase subunit TIM14; Probable component of the PAM complex, a complex required for the translocation of transit peptide-containing proteins from the inner membrane into the mitochondrial matrix in an ATP-dependent manner. May act as a co-chaperone that stimulate the ATP-dependent activity (By similarity); Belongs to the TIM14 family (116 aa) | |||
| DNAJC30 | DnaJ heat shock protein family member C30 (226 aa) | |||
| TIMM17B | Translocase of inner mitochondrial membrane 17B; TIM23 complex (222 aa) | |||
| HSBP1 | Heat shock factor-binding protein 1; Negative regulator of the heat shock response. Negatively affects HSF1 DNA-binding activity. May have a role in the suppression of the activation of the stress response during the aging process; Belongs to the HSBP1 family (76 aa) | |||
| DNAJC4 | DnaJ heat shock protein family member C4 (241 aa) | |||
| ENSG00000103426 | Coronin-7; F-actin regulator involved in anterograde Golgi to endosome transport- upon ubiquitination via ’Lys-33’-linked ubiquitin chains by the BCR(KLHL20) E3 ubiquitin ligase complex, interacts with EPS15 and localizes to the trans-Golgi network, where it promotes actin polymerization, thereby facilitating post- Golgi trafficking. May play a role in the maintenance of the Golgi apparatus morphology; Belongs to the WD repeat coronin family (1048 aa) | |||
| TIMM23 | Mitochondrial import inner membrane translocase subunit Tim23; Essential component of the TIM23 complex, a complex that mediates the translocation of transit peptide-containing proteins across the mitochondrial inner membrane; Belongs to the Tim17/Tim22/Tim23 family (209 aa) | |||
| HAO2 | Hydroxyacid oxidase 2; Catalyzes the oxidation of L-alpha-hydroxy acids as well as, more slowly, that of L-alpha-amino acids; Belongs to the FMN-dependent alpha-hydroxy acid dehydrogenase family (351 aa) | |||
