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Details on Person UniProt:O00483 COXFA4
| Class:Id | ReferenceGeneProduct:60618 |
|---|---|
| _chainChangeLog | chain:1-81 added on Fri February 6 2015 |
| _displayName | UniProt:O00483 COXFA4 |
| _timestamp | 2026-02-20 23:17:06 |
| chain | chain:1-81 |
| checksum | 2FA1D1115EDE24C7 |
| comment | FUNCTION Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules unsing 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix (PubMed:22902835). COXFA4 is required for complex IV maintenance (PubMed:22902835).SUBUNIT Component of the cytochrome c oxidase (complex IV, CIV), a multisubunit enzyme composed of 14 subunits. The complex is composed of a catalytic core of 3 subunits MT-CO1, MT-CO2 and MT-CO3, encoded in the mitochondrial DNA, and 11 supernumerary subunits COX4I1 (or COX4I2), COX5A, COX5B, COX6A1 (or COX6A2), COX6B1 (or COX6B2), COX6C, COX7A2 (or COX7A1), COX7B, COX7C, COX8A and COXFA4, which are encoded in the nuclear genome (PubMed:22902835, PubMed:23746447, PubMed:30030519). The complex exists as a monomer or a dimer and forms supercomplexes (SCs) in the inner mitochondrial membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII), resulting in different assemblies (supercomplex SCI(1)III(2)IV(1) and megacomplex MCI(2)III(2)IV(2)) (Probable). Interacts with RAB5IF (PubMed:31536960). Interacts with FLVCR2; this interaction occurs in the absence of heme and is disrupted upon heme binding.INTERACTION The disease may be caused by variants affecting the gene represented in this entry.MISCELLANEOUS During complex IV purifications dissociates from complex IV upon treatment with standard detergent DDM (decyl beta-D-maltoside) in high concentrations (PubMed:22902835, PubMed:23746447).SIMILARITY Belongs to the complex IV COXFA4 subunit family.CAUTION Was initially believed to be a subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). |
| description | recommendedName: fullName evidence="6"Cytochrome c oxidase subunit FA4 alternativeName: Complex I-MLRQ shortName: CI-MLRQ alternativeName: Cytochrome c oxidase subunit NDUFA4 alternativeName: NADH-ubiquinone oxidoreductase MLRQ subunit |
| geneName | COXFA4 NDUFA4 |
| identifier | O00483 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Electron transport Leigh syndrome Membrane Mitochondrion Mitochondrion inner membrane Phosphoprotein Primary mitochondrial disease Proteomics identification Reference proteome Respiratory chain Transmembrane Transmembrane helix Transport |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9841277] Weiser, Joel [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9983091] Weiser, Joel, 2026-02-20 |
| name | COXFA4 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8989084] ENSEMBL:ENSG00000189043 COXFA4 [Homo sapiens] |
| secondaryIdentifier | CXFA4_HUMAN A4D109 Q6FHN5 |
| sequenceLength | 81 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:60617] NDUFA4 [mitochondrial inner membrane] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:O00483 COXFA4 (60618)
