ID COX41_THUOB Reviewed; 169 AA. AC Q9I8U0; DT 03-APR-2002, integrated into UniProtKB/Swiss-Prot. DT 01-OCT-2000, sequence version 1. DT 22-FEB-2023, entry version 81. DE RecName: Full=Cytochrome c oxidase subunit 4 isoform 1, mitochondrial; DE AltName: Full=Cytochrome c oxidase subunit IV isoform 1; DE Short=COX IV-1; DE Flags: Precursor; OS Thunnus obesus (Bigeye tuna). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Actinopterygii; Neopterygii; Teleostei; Neoteleostei; Acanthomorphata; OC Pelagiaria; Scombriformes; Scombridae; Thunnus. OX NCBI_TaxID=8241; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA]. RC TISSUE=Heart, Liver, and Muscle; RX PubMed=11004496; DOI=10.1016/s0167-4781(00)00083-x; RA Huettemann M.; RT "New isoforms of cytochrome c oxidase subunit IV in tuna fish."; RL Biochim. Biophys. Acta 1492:242-246(2000). CC -!- FUNCTION: Component of the cytochrome c oxidase, the last enzyme in the CC mitochondrial electron transport chain which drives oxidative CC phosphorylation. The respiratory chain contains 3 multisubunit CC complexes succinate dehydrogenase (complex II, CII), ubiquinol- CC cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CC CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to CC transfer electrons derived from NADH and succinate to molecular oxygen, CC creating an electrochemical gradient over the inner membrane that CC drives transmembrane transport and the ATP synthase. Cytochrome c CC oxidase is the component of the respiratory chain that catalyzes the CC reduction of oxygen to water. Electrons originating from reduced CC cytochrome c in the intermembrane space (IMS) are transferred via the CC dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 CC to the active site in subunit 1, a binuclear center (BNC) formed by CC heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 CC water molecules using 4 electrons from cytochrome c in the IMS and 4 CC protons from the mitochondrial matrix. {ECO:0000250|UniProtKB:P00424}. CC -!- PATHWAY: Energy metabolism; oxidative phosphorylation. CC {ECO:0000250|UniProtKB:P00424}. CC -!- SUBUNIT: Component of the cytochrome c oxidase (complex IV, CIV), a CC multisubunit enzyme composed of 14 subunits. The complex is composed of CC a catalytic core of 3 subunits MT-CO1, MT-CO2 and MT-CO3, encoded in CC the mitochondrial DNA, and 11 supernumerary subunits COX4I, COX5A, CC COX5B, COX6A, COX6B, COX6C, COX7A, COX7B, COX7C, COX8 and NDUFA4, which CC are encoded in the nuclear genome. The complex exists as a monomer or a CC dimer and forms supercomplexes (SCs) in the inner mitochondrial CC membrane with NADH-ubiquinone oxidoreductase (complex I, CI) and CC ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex CC III, CIII), resulting in different assemblies (supercomplex CC SCI(1)III(2)IV(1) and megacomplex MCI(2)III(2)IV(2)). CC {ECO:0000250|UniProtKB:P00423}. CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane CC {ECO:0000250|UniProtKB:P00423}; Single-pass membrane protein CC {ECO:0000250|UniProtKB:P00423}. CC -!- SIMILARITY: Belongs to the cytochrome c oxidase IV family. CC {ECO:0000305}. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; AF204870; AAF79933.1; -; mRNA. DR AlphaFoldDB; Q9I8U0; -. DR SMR; Q9I8U0; -. DR UniPathway; UPA00705; -. DR GO; GO:0005751; C:mitochondrial respiratory chain complex IV; IEA:InterPro. DR GO; GO:0006123; P:mitochondrial electron transport, cytochrome c to oxygen; IEA:InterPro. DR CDD; cd00922; Cyt_c_Oxidase_IV; 1. DR Gene3D; 1.10.442.10; Cytochrome c oxidase subunit IV; 1. DR InterPro; IPR013288; Cyt_c_oxidase_su4. DR InterPro; IPR004203; Cyt_c_oxidase_su4_fam. DR InterPro; IPR036639; Cyt_c_oxidase_su4_sf. DR PANTHER; PTHR10707:SF14; CYTOCHROME C OXIDASE SUBUNIT 4; 1. DR PANTHER; PTHR10707; CYTOCHROME C OXIDASE SUBUNIT IV; 1. DR Pfam; PF02936; COX4; 1. DR PRINTS; PR01873; CYTCOXIDASE4. DR SUPFAM; SSF81406; Mitochondrial cytochrome c oxidase subunit IV; 1. PE 2: Evidence at transcript level; KW Membrane; Mitochondrion; Mitochondrion inner membrane; Transit peptide; KW Transmembrane; Transmembrane helix. FT TRANSIT 1..22 FT /note="Mitochondrion" FT /evidence="ECO:0000250" FT CHAIN 23..169 FT /note="Cytochrome c oxidase subunit 4 isoform 1, FT mitochondrial" FT /id="PRO_0000006092" FT TOPO_DOM 23..98 FT /note="Mitochondrial matrix" FT /evidence="ECO:0000250|UniProtKB:P00423" FT TRANSMEM 99..124 FT /note="Helical" FT /evidence="ECO:0000250|UniProtKB:P00423" FT TOPO_DOM 125..169 FT /note="Mitochondrial intermembrane" FT /evidence="ECO:0000250|UniProtKB:P00423" SQ SEQUENCE 169 AA; 19277 MW; F1ED066C2790FE0B CRC64; MLATGALSLV GKRAISTSVC LRGGHGVAKV EEYTLPAYFD RRENPLPDIC YVQALSPEQQ SLKEKEKGSW AALSNEEKIA LYRISFKQSF AEMNKGSSEW KSVVGGMLFF IGFTGLVVLW QRKYVYGAVP HTFDPEYKAK ELQRMLDMRI NPVEGFSAHW DYENKQWKK //