UniProtKB - P20674 (COX5A_HUMAN)
Cytochrome c oxidase subunit 5A, mitochondrial
COX5A
Functioni
: oxidative phosphorylation Pathwayi
This protein is involved in the pathway oxidative phosphorylation, which is part of Energy metabolism.By similarityView all proteins of this organism that are known to be involved in the pathway oxidative phosphorylation and in Energy metabolism.
GO - Molecular functioni
- cytochrome-c oxidase activity Source: GO_Central
- electron transfer activity Source: UniProtKB
- metal ion binding Source: UniProtKB-KW
GO - Biological processi
- mitochondrial electron transport, cytochrome c to oxygen Source: GO_Central
Keywordsi
Ligand | Heme, Iron, Metal-binding |
Enzyme and pathway databases
BioCyci | MetaCyc:HS11312-MONOMER |
PathwayCommonsi | P20674 |
Reactomei | R-HSA-5628897, TP53 Regulates Metabolic Genes R-HSA-611105, Respiratory electron transport |
UniPathwayi | UPA00705 |
Protein family/group databases
TCDBi | 3.D.4.11.1, the proton-translocating cytochrome oxidase (cox) superfamily |
Names & Taxonomyi
Protein namesi | Recommended name: Cytochrome c oxidase subunit 5A, mitochondrialAlternative name(s): Cytochrome c oxidase polypeptide Va |
Gene namesi | Name:COX5A |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
EuPathDBi | HostDB:ENSG00000178741.11 |
HGNCi | HGNC:2267, COX5A |
MIMi | 603773, gene |
neXtProti | NX_P20674 |
Subcellular locationi
Mitochondrion
- Mitochondrion inner membrane 1 Publication; Peripheral membrane protein 1 Publication; Matrix side 1 Publication
Mitochondrion
- mitochondrial inner membrane Source: CAFA
- mitochondrial respiratory chain complex IV Source: UniProtKB
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion inner membranePathology & Biotechi
Involvement in diseasei
Keywords - Diseasei
Disease mutation, Primary mitochondrial diseaseOrganism-specific databases
DisGeNETi | 9377 |
MalaCardsi | COX5A |
OpenTargetsi | ENSG00000178741 |
Orphaneti | 254905, Isolated cytochrome C oxidase deficiency |
PharmGKBi | PA26784 |
Miscellaneous databases
Pharosi | P20674, Tbio |
Chemistry databases
DrugBanki | DB02659, Cholic Acid DB04464, N-Formylmethionine |
Polymorphism and mutation databases
BioMutai | COX5A |
DMDMi | 218511986 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 41 | MitochondrionCombined sources1 PublicationAdd BLAST | 41 | |
ChainiPRO_0000006100 | 42 – 150 | Cytochrome c oxidase subunit 5A, mitochondrialAdd BLAST | 109 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 87 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 113 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 141 | PhosphothreonineCombined sources | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
EPDi | P20674 |
jPOSTi | P20674 |
MassIVEi | P20674 |
MaxQBi | P20674 |
PaxDbi | P20674 |
PeptideAtlasi | P20674 |
PRIDEi | P20674 |
ProteomicsDBi | 53772 |
TopDownProteomicsi | P20674 |
2D gel databases
DOSAC-COBS-2DPAGEi | P20674 |
OGPi | P20674 |
SWISS-2DPAGEi | P20674 |
UCD-2DPAGEi | P20674 |
PTM databases
iPTMneti | P20674 |
PhosphoSitePlusi | P20674 |
SwissPalmi | P20674 |
Expressioni
Gene expression databases
Bgeei | ENSG00000178741, Expressed in heart left ventricle and 246 other tissues |
ExpressionAtlasi | P20674, baseline and differential |
Genevisiblei | P20674, HS |
Organism-specific databases
HPAi | ENSG00000178741, Tissue enhanced (heart muscle, skeletal muscle) |
Interactioni
Subunit structurei
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 NDUFA4, which are encoded in the nuclear genome (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 SCI1III2IV1 and megacomplex MCI2III2IV2) (PubMed:28844695).
Interacts with AFG1L (PubMed:26759378).
Interacts with RAB5IF (PubMed:31536960).
4 PublicationsBinary interactionsi
Hide detailsP20674
Protein-protein interaction databases
BioGRIDi | 114778, 125 interactors |
CORUMi | P20674 |
IntActi | P20674, 56 interactors |
MINTi | P20674 |
STRINGi | 9606.ENSP00000317780 |
Miscellaneous databases
RNActi | P20674, protein |
Family & Domainsi
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG4077, Eukaryota |
GeneTreei | ENSGT00390000001424 |
HOGENOMi | CLU_099086_1_1_1 |
InParanoidi | P20674 |
OMAi | DIDHWEI |
OrthoDBi | 1286549at2759 |
PhylomeDBi | P20674 |
TreeFami | TF105062 |
Family and domain databases
CDDi | cd00923, Cyt_c_Oxidase_Va, 1 hit |
Gene3Di | 1.25.40.40, 1 hit |
InterProi | View protein in InterPro IPR003204, Cyt_c_oxidase_su5A/6 IPR036545, Cyt_c_oxidase_su5A/6_sf |
PANTHERi | PTHR14200, PTHR14200, 1 hit |
Pfami | View protein in Pfam PF02284, COX5A, 1 hit |
SUPFAMi | SSF48479, SSF48479, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show allAlign All
10 20 30 40 50
MLGAALRRCA VAATTRADPR GLLHSARTPG PAVAIQSVRC YSHGSQETDE
60 70 80 90 100
EFDARWVTYF NKPDIDAWEL RKGINTLVTY DMVPEPKIID AALRACRRLN
110 120 130 140 150
DFASTVRILE VVKDKAGPHK EIYPYVIQEL RPTLNELGIS TPEELGLDKV
Computationally mapped potential isoform sequencesi
There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketH3BNX8 | H3BNX8_HUMAN | Cytochrome c oxidase polypeptide Va | COX5A | 153 | Annotation score: | ||
H3BRM5 | H3BRM5_HUMAN | Cytochrome c oxidase polypeptide Va | COX5A | 69 | Annotation score: | ||
H3BV69 | H3BV69_HUMAN | Cytochrome c oxidase polypeptide Va | COX5A | 111 | Annotation score: | ||
H3BRI0 | H3BRI0_HUMAN | Cytochrome c oxidase polypeptide Va | COX5A | 84 | Annotation score: |
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 105 | T → L in AAA99220 (PubMed:2853101).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_078264 | 107 | R → C Probable disease-associated variant found in patients with mitochondrial complex IV deficiency; decreased protein abundance in patient fibroblasts; decreased proteins abundance of mitochondrial respiratory chain complex IV in patient fibroblasts; no effect on respiratory chain complex IV assembly in patient fibroblasts; increased protein abundance of S1 complex IV intermediate in patient fibroblasts. 1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M22760 mRNA Translation: AAA99220.1 DQ987236 mRNA Translation: ABK92283.1 DQ987237 mRNA Translation: ABK92284.1 CR407649 mRNA Translation: CAG28577.1 AC125435 Genomic DNA No translation available. CH471136 Genomic DNA Translation: EAW99288.1 BC024240 mRNA Translation: AAH24240.1 |
CCDSi | CCDS10273.1 |
PIRi | JT0342, OTHU5A |
RefSeqi | NP_004246.2, NM_004255.3 |
Genome annotation databases
Ensembli | ENST00000322347; ENSP00000317780; ENSG00000178741 ENST00000564811; ENSP00000456386; ENSG00000178741 |
GeneIDi | 9377 |
KEGGi | hsa:9377 |
UCSCi | uc002azi.5, human |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | M22760 mRNA Translation: AAA99220.1 DQ987236 mRNA Translation: ABK92283.1 DQ987237 mRNA Translation: ABK92284.1 CR407649 mRNA Translation: CAG28577.1 AC125435 Genomic DNA No translation available. CH471136 Genomic DNA Translation: EAW99288.1 BC024240 mRNA Translation: AAH24240.1 |
CCDSi | CCDS10273.1 |
PIRi | JT0342, OTHU5A |
RefSeqi | NP_004246.2, NM_004255.3 |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
5Z62 | electron microscopy | 3.60 | E | 42-150 | [»] | |
SMRi | P20674 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 114778, 125 interactors |
CORUMi | P20674 |
IntActi | P20674, 56 interactors |
MINTi | P20674 |
STRINGi | 9606.ENSP00000317780 |
Chemistry databases
DrugBanki | DB02659, Cholic Acid DB04464, N-Formylmethionine |
Protein family/group databases
TCDBi | 3.D.4.11.1, the proton-translocating cytochrome oxidase (cox) superfamily |
PTM databases
iPTMneti | P20674 |
PhosphoSitePlusi | P20674 |
SwissPalmi | P20674 |
Polymorphism and mutation databases
BioMutai | COX5A |
DMDMi | 218511986 |
2D gel databases
DOSAC-COBS-2DPAGEi | P20674 |
OGPi | P20674 |
SWISS-2DPAGEi | P20674 |
UCD-2DPAGEi | P20674 |
Proteomic databases
EPDi | P20674 |
jPOSTi | P20674 |
MassIVEi | P20674 |
MaxQBi | P20674 |
PaxDbi | P20674 |
PeptideAtlasi | P20674 |
PRIDEi | P20674 |
ProteomicsDBi | 53772 |
TopDownProteomicsi | P20674 |
Protocols and materials databases
Antibodypediai | 27166, 338 antibodies |
DNASUi | 9377 |
Genome annotation databases
Ensembli | ENST00000322347; ENSP00000317780; ENSG00000178741 ENST00000564811; ENSP00000456386; ENSG00000178741 |
GeneIDi | 9377 |
KEGGi | hsa:9377 |
UCSCi | uc002azi.5, human |
Organism-specific databases
CTDi | 9377 |
DisGeNETi | 9377 |
EuPathDBi | HostDB:ENSG00000178741.11 |
GeneCardsi | COX5A |
HGNCi | HGNC:2267, COX5A |
HPAi | ENSG00000178741, Tissue enhanced (heart muscle, skeletal muscle) |
MalaCardsi | COX5A |
MIMi | 603773, gene |
neXtProti | NX_P20674 |
OpenTargetsi | ENSG00000178741 |
Orphaneti | 254905, Isolated cytochrome C oxidase deficiency |
PharmGKBi | PA26784 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG4077, Eukaryota |
GeneTreei | ENSGT00390000001424 |
HOGENOMi | CLU_099086_1_1_1 |
InParanoidi | P20674 |
OMAi | DIDHWEI |
OrthoDBi | 1286549at2759 |
PhylomeDBi | P20674 |
TreeFami | TF105062 |
Enzyme and pathway databases
UniPathwayi | UPA00705 |
BioCyci | MetaCyc:HS11312-MONOMER |
PathwayCommonsi | P20674 |
Reactomei | R-HSA-5628897, TP53 Regulates Metabolic Genes R-HSA-611105, Respiratory electron transport |
Miscellaneous databases
BioGRID-ORCSi | 9377, 393 hits in 845 CRISPR screens |
ChiTaRSi | COX5A, human |
GenomeRNAii | 9377 |
Pharosi | P20674, Tbio |
PROi | PR:P20674 |
RNActi | P20674, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000178741, Expressed in heart left ventricle and 246 other tissues |
ExpressionAtlasi | P20674, baseline and differential |
Genevisiblei | P20674, HS |
Family and domain databases
CDDi | cd00923, Cyt_c_Oxidase_Va, 1 hit |
Gene3Di | 1.25.40.40, 1 hit |
InterProi | View protein in InterPro IPR003204, Cyt_c_oxidase_su5A/6 IPR036545, Cyt_c_oxidase_su5A/6_sf |
PANTHERi | PTHR14200, PTHR14200, 1 hit |
Pfami | View protein in Pfam PF02284, COX5A, 1 hit |
SUPFAMi | SSF48479, SSF48479, 1 hit |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | COX5A_HUMAN | |
Accessioni | P20674Primary (citable) accession number: P20674 Secondary accession number(s): P30045, Q8TB65 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | February 1, 1991 |
Last sequence update: | December 16, 2008 | |
Last modified: | December 2, 2020 | |
This is version 187 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
3D-structure, Direct protein sequencing, Reference proteomeDocuments
- Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot - PATHWAY comments
Index of metabolic and biosynthesis pathways - PDB cross-references
Index of Protein Data Bank (PDB) cross-references - SIMILARITY comments
Index of protein domains and families - Human chromosome 15
Human chromosome 15: entries, gene names and cross-references to MIM - Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations