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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) | |||
| COX6A1 | Cytochrome c oxidase subunit 6A1, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (109 aa) | |||
| PMPCB | Mitochondrial-processing peptidase subunit beta; Cleaves presequences (transit peptides) from mitochondrial protein precursors; M16 metallopeptidases (489 aa) | |||
| COX5B | Cytochrome c oxidase subunit 5B, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (129 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) | |||
| HIGD2A | HIG1 domain family member 2A, mitochondrial; Proposed subunit of cytochrome c oxidase (COX, complex IV), which is the terminal component of the mitochondrial respiratory chain that catalyzes the reduction of oxygen to water. May be involved in cytochrome c oxidase activity. May play a role in the assembly of respiratory supercomplexes (106 aa) | |||
| COX6A2 | Cytochrome c oxidase subunit 6A2, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (97 aa) | |||
| UQCRFS1 | Cytochrome b-c1 complex subunit Rieske, mitochondrial; Cytochrome b-c1 complex subunit Rieske, mitochondrial- Component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis. Incorporation of UQCRFS1 is the penultimate step in complex III assembly (By similarity) (274 aa) | |||
| SFXN1 | Sideroflexin-1; Might be involved in the transport of a component required for iron utilization into or out of the mitochondria; Sideroflexins (322 aa) | |||
| CYC1 | Cytochrome c1, heme protein, mitochondrial; This is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain; Apoptosome (325 aa) | |||
| MINOS1 | MICOS complex subunit MIC10; Component of the MICOS complex, a large protein complex of the mitochondrial inner membrane that plays crucial roles in the maintenance of crista junctions, inner membrane architecture, and formation of contact sites to the outer membrane (78 aa) | |||
| FIP1L1 | Pre-mRNA 3’-end-processing factor FIP1; Component of the cleavage and polyadenylation specificity factor (CPSF) complex that plays a key role in pre- mRNA 3’-end formation, recognizing the AAUAAA signal sequence and interacting with poly(A) polymerase and other factors to bring about cleavage and poly(A) addition. FIP1L1 contributes to poly(A) site recognition and stimulates poly(A) addition. Binds to U-rich RNA sequence elements surrounding the poly(A) site. May act to tether poly(A) polymerase to the CPSF complex (594 aa) | |||
| PCDHB11 | Protocadherin beta-11; Potential calcium-dependent cell-adhesion protein. May be involved in the establishment and maintenance of specific neuronal connections in the brain; Clustered protocadherins (797 aa) | |||
| MT-CO1 | Cytochrome c oxidase subunit 1; Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. CO I is the catalytic subunit of the enzyme. Electrons originating in cytochrome c are transferred via the copper A center of subunit 2 and heme A of subunit 1 to the bimetallic center formed by heme A3 and copper B (513 aa) | |||
| MT-CYB | Cytochrome b; Component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex) that is part of the mitochondrial respiratory chain. The b-c1 complex mediates electron transfer from ubiquinol to cytochrome c. Contributes to the generation of a proton gradient across the mitochondrial membrane that is then used for ATP synthesis (380 aa) | |||
| MT-CO2 | Cytochrome c oxidase subunit 2; Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1- 3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1 (227 aa) | |||
| MT-CO3 | Cytochrome c oxidase subunit 3; Subunits I, II and III form the functional core of the enzyme complex; Mitochondrial complex IV- cytochrome c oxidase subunits (261 aa) | |||
| PEX19 | Peroxisomal biogenesis factor 19; Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53; Belongs to the peroxin-19 family (299 aa) | |||
| SLC25A5 | ADP/ATP translocase 2; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. As part of the mitotic spindle-associated MMXD complex it may play a role in chromosome segregation; Solute carriers (298 aa) | |||
| PMPCA | Mitochondrial-processing peptidase subunit alpha; Cleaves presequences (transit peptides) from mitochondrial protein precursors; Belongs to the peptidase M16 family (525 aa) | |||
| COX4I2 | Cytochrome c oxidase subunit 4 isoform 2, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (171 aa) | |||
| CRLS1 | Cardiolipin synthase (CMP-forming); Catalyzes the synthesis of cardiolipin (CL) (diphosphatidylglycerol) by specifically transferring a phosphatidyl group from CDP-diacylglycerol to phosphatidylglycerol (PG). CL is a key phospholipid in mitochondrial membranes and plays important roles in maintaining the functional integrity and dynamics of mitochondria under both optimal and stress conditions (301 aa) | |||
| SLC25A6 | ADP/ATP translocase 3; Catalyzes the exchange of cytoplasmic ADP with mitochondrial ATP across the mitochondrial inner membrane. May participate in the formation of the permeability transition pore complex (PTPC) responsible for the release of mitochondrial products that triggers apoptosis; Belongs to the mitochondrial carrier (TC 2.A.29) family (298 aa) | |||
| ENSG00000111780 | Uncharacterized protein; Glutamyl-tRNA(Gln) amidotransferase subunit C, mitochondrial (168 aa) | |||
| COX4I1 | Cytochrome c oxidase subunit 4 isoform 1, mitochondrial; This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport (169 aa) | |||
| PHB | Prohibitin; Prohibitin inhibits DNA synthesis. It has a role in regulating proliferation. As yet it is unclear if the protein or the mRNA exhibits this effect. May play a role in regulating mitochondrial respiration activity and in aging (272 aa) | |||
