ID CX7A1_HUMAN Reviewed; 79 AA. AC P24310; DT 01-MAR-1992, integrated into UniProtKB/Swiss-Prot. DT 01-JUL-1993, sequence version 2. DT 24-JAN-2024, entry version 185. DE RecName: Full=Cytochrome c oxidase subunit 7A1, mitochondrial; DE AltName: Full=Cytochrome c oxidase subunit VIIa-heart; DE Short=Cytochrome c oxidase subunit VIIa-H; DE AltName: Full=Cytochrome c oxidase subunit VIIa-muscle; DE Short=Cytochrome c oxidase subunit VIIa-M; DE Flags: Precursor; GN Name=COX7A1; Synonyms=COX7AH; OS Homo sapiens (Human). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; OC Homo. OX NCBI_TaxID=9606; RN [1] RP NUCLEOTIDE SEQUENCE [MRNA]. RX PubMed=1327965; DOI=10.1016/0378-1119(92)90287-y; RA Arnaudo E., Hirano M., Seelan R.S., Milatovich A., Hsieh C.L., RA Fabrizi G.M., Grossman L.I., Francke U., Schon E.A.; RT "Tissue-specific expression and chromosome assignment of genes specifying RT two isoforms of subunit VIIa of human cytochrome c oxidase."; RL Gene 119:299-305(1992). RN [2] RP NUCLEOTIDE SEQUENCE [GENOMIC DNA]. RX PubMed=9344674; DOI=10.1006/geno.1997.4937; RA Wolz W., Kress W., Mueller C.R.; RT "Genomic sequence and organization of the human gene for cytochrome c RT oxidase subunit (COX7A1) VIIa-M."; RL Genomics 45:438-442(1997). RN [3] RP NUCLEOTIDE SEQUENCE [GENOMIC DNA]. RC TISSUE=Heart; RX PubMed=11997101; DOI=10.1016/s0167-4781(02)00228-2; RA Yu M., Jaradat S.A., Grossman L.I.; RT "Genomic organization and promoter regulation of human cytochrome c oxidase RT subunit VII heart/muscle isoform (COX7AH)."; RL Biochim. Biophys. Acta 1574:345-353(2002). RN [4] RP NUCLEOTIDE SEQUENCE [GENOMIC DNA]. RX PubMed=10335655; DOI=10.1093/oxfordjournals.molbev.a026144; RA Schmidt T.R., Goodman M., Grossman L.I.; RT "Molecular evolution of the COX7A gene family in primates."; RL Mol. Biol. Evol. 16:619-626(1999). RN [5] RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. RX PubMed=15057824; DOI=10.1038/nature02399; RA Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., Lamerdin J.E., RA Hellsten U., Goodstein D., Couronne O., Tran-Gyamfi M., Aerts A., RA Altherr M., Ashworth L., Bajorek E., Black S., Branscomb E., Caenepeel S., RA Carrano A.V., Caoile C., Chan Y.M., Christensen M., Cleland C.A., RA Copeland A., Dalin E., Dehal P., Denys M., Detter J.C., Escobar J., RA Flowers D., Fotopulos D., Garcia C., Georgescu A.M., Glavina T., Gomez M., RA Gonzales E., Groza M., Hammon N., Hawkins T., Haydu L., Ho I., Huang W., RA Israni S., Jett J., Kadner K., Kimball H., Kobayashi A., Larionov V., RA Leem S.-H., Lopez F., Lou Y., Lowry S., Malfatti S., Martinez D., RA McCready P.M., Medina C., Morgan J., Nelson K., Nolan M., Ovcharenko I., RA Pitluck S., Pollard M., Popkie A.P., Predki P., Quan G., Ramirez L., RA Rash S., Retterer J., Rodriguez A., Rogers S., Salamov A., Salazar A., RA She X., Smith D., Slezak T., Solovyev V., Thayer N., Tice H., Tsai M., RA Ustaszewska A., Vo N., Wagner M., Wheeler J., Wu K., Xie G., Yang J., RA Dubchak I., Furey T.S., DeJong P., Dickson M., Gordon D., Eichler E.E., RA Pennacchio L.A., Richardson P., Stubbs L., Rokhsar D.S., Myers R.M., RA Rubin E.M., Lucas S.M.; RT "The DNA sequence and biology of human chromosome 19."; RL Nature 428:529-535(2004). RN [6] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. RC TISSUE=Uterus; RX PubMed=15489334; DOI=10.1101/gr.2596504; RG The MGC Project Team; RT "The status, quality, and expansion of the NIH full-length cDNA project: RT the Mammalian Gene Collection (MGC)."; RL Genome Res. 14:2121-2127(2004). RN [7] RP PROTEIN SEQUENCE OF 22-51. RC TISSUE=Heart, and Skeletal muscle; RX PubMed=1309697; DOI=10.1111/j.1432-1033.1992.tb19847.x; RA van Kuilenburg A.B.P., van Beeumen J.J., van der Meer N.M., Muijsers A.O.; RT "Subunits VIIa,b,c of human cytochrome c oxidase. Identification of both RT 'heart-type' and 'liver-type' isoforms of subunit VIIa in human heart."; RL Eur. J. Biochem. 203:193-199(1992). 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:P10174}. CC -!- PATHWAY: Energy metabolism; oxidative phosphorylation. CC {ECO:0000250|UniProtKB:P10174}. 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 COX4I1 (or CC COX4I2), COX5A, COX5B, COX6A1 (or COX6A2), COX6B1 (or COX6B2), COX6C, CC COX7A2 (or COX7A1), COX7B, COX7C, COX8A and NDUFA4, which are encoded CC in the nuclear genome (By similarity). The complex exists as a monomer CC or a 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)) (By similarity). CC {ECO:0000250|UniProtKB:P07470}. CC -!- INTERACTION: CC P24310; O75190-2: DNAJB6; NbExp=3; IntAct=EBI-25876196, EBI-12593112; CC P24310; O14901: KLF11; NbExp=3; IntAct=EBI-25876196, EBI-948266; CC P24310; P28331-2: NDUFS1; NbExp=3; IntAct=EBI-25876196, EBI-6190702; CC P24310; Q9BVL2: NUP58; NbExp=3; IntAct=EBI-25876196, EBI-2811583; CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane CC {ECO:0000250|UniProtKB:P07470}; Single-pass membrane protein CC {ECO:0000250|UniProtKB:P07470}. CC -!- SIMILARITY: Belongs to the cytochrome c oxidase VIIa 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; M83186; AAA52166.1; -; mRNA. DR EMBL; U81524; AAB82735.1; -; Genomic_DNA. DR EMBL; AC002984; AAB81547.1; -; Genomic_DNA. DR EMBL; AF037372; AAB92616.1; -; Genomic_DNA. DR EMBL; AF127789; AAF72747.1; -; Genomic_DNA. DR EMBL; AD001527; AAB51184.1; -; Genomic_DNA. DR EMBL; BC002757; AAH02757.1; -; mRNA. DR CCDS; CCDS12490.1; -. DR PIR; JC1303; OSHU7A. DR RefSeq; NP_001855.1; NM_001864.3. DR AlphaFoldDB; P24310; -. DR SMR; P24310; -. DR BioGRID; 107739; 11. DR IntAct; P24310; 4. DR STRING; 9606.ENSP00000292907; -. DR DrugBank; DB02659; Cholic Acid. DR DrugBank; DB04464; N-Formylmethionine. DR TCDB; 3.D.4.11.1; the proton-translocating cytochrome oxidase (cox) superfamily. DR iPTMnet; P24310; -. DR PhosphoSitePlus; P24310; -. DR SwissPalm; P24310; -. DR BioMuta; COX7A1; -. DR jPOST; P24310; -. DR MassIVE; P24310; -. DR PaxDb; 9606-ENSP00000292907; -. DR PeptideAtlas; P24310; -. DR ProteomicsDB; 54195; -. DR Antibodypedia; 44654; 123 antibodies from 22 providers. DR DNASU; 1346; -. DR Ensembl; ENST00000292907.8; ENSP00000292907.3; ENSG00000161281.11. DR GeneID; 1346; -. DR KEGG; hsa:1346; -. DR MANE-Select; ENST00000292907.8; ENSP00000292907.3; NM_001864.4; NP_001855.1. DR AGR; HGNC:2287; -. DR CTD; 1346; -. DR DisGeNET; 1346; -. DR GeneCards; COX7A1; -. DR HGNC; HGNC:2287; COX7A1. DR HPA; ENSG00000161281; Group enriched (heart muscle, skeletal muscle, tongue). DR MIM; 123995; gene. DR neXtProt; NX_P24310; -. DR OpenTargets; ENSG00000161281; -. DR PharmGKB; PA26805; -. DR VEuPathDB; HostDB:ENSG00000161281; -. DR eggNOG; ENOG502SBK9; Eukaryota. DR GeneTree; ENSGT00940000162305; -. DR HOGENOM; CLU_173437_1_0_1; -. DR InParanoid; P24310; -. DR OMA; IAGTCYS; -. DR OrthoDB; 5352834at2759; -. DR PhylomeDB; P24310; -. DR TreeFam; TF105067; -. DR PathwayCommons; P24310; -. DR SignaLink; P24310; -. DR UniPathway; UPA00705; -. DR BioGRID-ORCS; 1346; 16 hits in 1158 CRISPR screens. DR ChiTaRS; COX7A1; human. DR GeneWiki; COX7A1; -. DR GenomeRNAi; 1346; -. DR Pharos; P24310; Tbio. DR PRO; PR:P24310; -. DR Proteomes; UP000005640; Chromosome 19. DR RNAct; P24310; Protein. DR Bgee; ENSG00000161281; Expressed in heart right ventricle and 196 other cell types or tissues. DR ExpressionAtlas; P24310; baseline and differential. DR GO; GO:0005746; C:mitochondrial respirasome; IBA:GO_Central. DR GO; GO:0005751; C:mitochondrial respiratory chain complex IV; IEA:InterPro. DR GO; GO:0005739; C:mitochondrion; TAS:ProtInc. DR GO; GO:0004129; F:cytochrome-c oxidase activity; TAS:ProtInc. DR GO; GO:0006091; P:generation of precursor metabolites and energy; TAS:ProtInc. DR GO; GO:0006123; P:mitochondrial electron transport, cytochrome c to oxygen; IEA:InterPro. DR GO; GO:0097250; P:mitochondrial respirasome assembly; IBA:GO_Central. DR GO; GO:0002082; P:regulation of oxidative phosphorylation; IBA:GO_Central. DR CDD; cd00928; Cyt_c_Oxidase_VIIa; 1. DR Gene3D; 4.10.91.10; Cytochrome c oxidase, subunit VIIa; 1. DR InterPro; IPR039297; COX7a. DR InterPro; IPR036539; Cyt_c_oxidase_su7a_sf. DR InterPro; IPR003177; Cytc_oxidase_su7a_met. DR PANTHER; PTHR10510; CYTOCHROME C OXIDASE POLYPEPTIDE 7A; 1. DR PANTHER; PTHR10510:SF5; CYTOCHROME C OXIDASE SUBUNIT 7A1, MITOCHONDRIAL; 1. DR Pfam; PF02238; COX7a; 1. DR SUPFAM; SSF81419; Mitochondrial cytochrome c oxidase subunit VIIa; 1. DR Genevisible; P24310; HS. PE 1: Evidence at protein level; KW Direct protein sequencing; Membrane; Mitochondrion; KW Mitochondrion inner membrane; Oxidoreductase; Reference proteome; KW Transit peptide; Transmembrane; Transmembrane helix. FT TRANSIT 1..21 FT /note="Mitochondrion" FT /evidence="ECO:0000269|PubMed:1309697" FT CHAIN 22..79 FT /note="Cytochrome c oxidase subunit 7A1, mitochondrial" FT /id="PRO_0000006149" FT TOPO_DOM 22..46 FT /note="Mitochondrial matrix" FT /evidence="ECO:0000250|UniProtKB:P07470" FT TRANSMEM 47..75 FT /note="Helical" FT /evidence="ECO:0000250|UniProtKB:P07470" FT TOPO_DOM 76..79 FT /note="Mitochondrial intermembrane" FT /evidence="ECO:0000250|UniProtKB:P07470" SQ SEQUENCE 79 AA; 9118 MW; AFE9C00C53CE35C2 CRC64; MQALRVSQAL IRSFSSTARN RFQNRVREKQ KLFQEDNDIP LYLKGGIVDN ILYRVTMTLC LGGTVYSLYS LGWASFPRN //