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Protein

Cytochrome c oxidase subunit 5A, mitochondrial

Gene

COX5A

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

This is the heme A-containing chain of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport.

GO - Molecular functioni

  • cytochrome-c oxidase activity Source: GO_Central
  • electron transfer activity Source: UniProtKB
  • metal ion binding Source: UniProtKB-KW

GO - Biological processi

Keywordsi

LigandHeme, Iron, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport

Protein family/group databases

TCDBi3.D.4.11.1 the proton-translocating cytochrome oxidase (cox) superfamily

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome c oxidase subunit 5A, mitochondrial
Alternative name(s):
Cytochrome c oxidase polypeptide Va
Gene namesi
Name:COX5A
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 15

Organism-specific databases

EuPathDBiHostDB:ENSG00000178741.11
HGNCiHGNC:2267 COX5A
MIMi603773 gene
neXtProtiNX_P20674

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Involvement in diseasei

Mitochondrial complex IV deficiency is a rare condition caused by mutation in COX5A that lead to pulmonary arterial hypertension (PAH), failure to thrive and lactic acidemia.1 Publication

Keywords - Diseasei

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNETi9377
MalaCardsiCOX5A
OpenTargetsiENSG00000178741
PharmGKBiPA26784

Chemistry databases

DrugBankiDB02659 Cholic Acid
DB04464 N-Formylmethionine

Polymorphism and mutation databases

BioMutaiCOX5A
DMDMi218511986

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 41MitochondrionCombined sources1 PublicationAdd BLAST41
ChainiPRO_000000610042 – 150Cytochrome c oxidase subunit 5A, mitochondrialAdd BLAST109

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei87N6-acetyllysineBy similarity1
Modified residuei113N6-acetyllysineBy similarity1
Modified residuei141PhosphothreonineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP20674
MaxQBiP20674
PaxDbiP20674
PeptideAtlasiP20674
PRIDEiP20674
TopDownProteomicsiP20674

2D gel databases

DOSAC-COBS-2DPAGEiP20674
OGPiP20674
SWISS-2DPAGEiP20674
UCD-2DPAGEiP20674

PTM databases

iPTMnetiP20674
PhosphoSitePlusiP20674
SwissPalmiP20674

Expressioni

Gene expression databases

BgeeiENSG00000178741
CleanExiHS_COX5A
ExpressionAtlasiP20674 baseline and differential
GenevisibleiP20674 HS

Organism-specific databases

HPAiHPA027526

Interactioni

Subunit structurei

Interacts with AFG1L (PubMed:26759378).1 Publication

Binary interactionsi

Show more details

Protein-protein interaction databases

BioGridi114778, 59 interactors
CORUMiP20674
IntActiP20674, 33 interactors
STRINGi9606.ENSP00000317780

Structurei

3D structure databases

ProteinModelPortaliP20674
SMRiP20674
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG4077 Eukaryota
ENOG4111MPD LUCA
GeneTreeiENSGT00390000001424
HOGENOMiHOG000160656
HOVERGENiHBG051088
InParanoidiP20674
KOiK02264
OMAiLNNCFAH
PhylomeDBiP20674
TreeFamiTF105062

Family and domain databases

CDDicd00923 Cyt_c_Oxidase_Va, 1 hit
Gene3Di1.25.40.40, 1 hit
InterProiView protein in InterPro
IPR003204 Cyt_c_oxidase_su5A/6
IPR036545 Cyt_c_oxidase_su5A/6_sf
PANTHERiPTHR14200 PTHR14200, 1 hit
PfamiView protein in Pfam
PF02284 COX5A, 1 hit
SUPFAMiSSF48479 SSF48479, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P20674-1 [UniParc]FASTAAdd to basket

« Hide

        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
Length:150
Mass (Da):16,762
Last modified:December 16, 2008 - v2
Checksum:i104DAF468BD302FC
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti105T → L in AAA99220 (PubMed:2853101).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_078264107R → C Probable disease-associated mutation 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 Publication1

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
CCDSiCCDS10273.1
PIRiJT0342 OTHU5A
RefSeqiNP_004246.2, NM_004255.3
UniGeneiHs.401903

Genome annotation databases

EnsembliENST00000322347; ENSP00000317780; ENSG00000178741
ENST00000564811; ENSP00000456386; ENSG00000178741
GeneIDi9377
KEGGihsa:9377
UCSCiuc002azi.5 human

Similar proteinsi

Entry informationi

Entry nameiCOX5A_HUMAN
AccessioniPrimary (citable) accession number: P20674
Secondary accession number(s): P30045, Q8TB65
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1991
Last sequence update: December 16, 2008
Last modified: May 23, 2018
This is version 168 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 15
    Human chromosome 15: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

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