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Protein

Cytochrome c oxidase assembly protein COX19

Gene

COX19

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

Functioni

Required for the transduction of an SCO1-dependent redox signal from the mitochondrion to ATP7A to regulate cellular copper homeostasis (PubMed:23345593). May be required for the assembly of mitochondrial cytochrome c oxidase (By similarity).By similarity1 Publication

GO - Biological processi

  • aerobic respiration Source: GO_Central
  • cellular copper ion homeostasis Source: UniProtKB
  • mitochondrial respiratory chain complex IV assembly Source: GO_Central

Enzyme and pathway databases

ReactomeiR-HSA-1268020 Mitochondrial protein import

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome c oxidase assembly protein COX19
Short name:
hCOX19
Gene namesi
Name:COX19
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

EuPathDBiHostDB:ENSG00000240230.5
HGNCiHGNC:28074 COX19
MIMi610429 gene
neXtProtiNX_Q49B96

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

Cytoplasm, Mitochondrion

Pathology & Biotechi

Organism-specific databases

DisGeNETi90639
OpenTargetsiENSG00000240230
PharmGKBiPA145008561

Polymorphism and mutation databases

BioMutaiCOX19
DMDMi121943561

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedCombined sources
ChainiPRO_00002731512 – 90Cytochrome c oxidase assembly protein COX19Add BLAST89

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylserineCombined sources1
Disulfide bondi30 ↔ 61PROSITE-ProRule annotation1 Publication
Disulfide bondi40 ↔ 51PROSITE-ProRule annotation1 Publication

Keywords - PTMi

Acetylation, Disulfide bond

Proteomic databases

EPDiQ49B96
MaxQBiQ49B96
PaxDbiQ49B96
PeptideAtlasiQ49B96
PRIDEiQ49B96

PTM databases

iPTMnetiQ49B96
PhosphoSitePlusiQ49B96

Expressioni

Tissue specificityi

Ubiquitously expressed. Highly expressed in skeletal muscle.1 Publication

Gene expression databases

BgeeiENSG00000240230
CleanExiHS_COX19
ExpressionAtlasiQ49B96 baseline and differential
GenevisibleiQ49B96 HS

Organism-specific databases

HPAiHPA021226

Interactioni

Subunit structurei

Interacts with CHCHD4/MIA40 forming transient intermolecular disulfide bridges.1 Publication

Protein-protein interaction databases

BioGridi1247481 interactor.
STRINGi9606.ENSP00000342015

Structurei

3D structure databases

ProteinModelPortaliQ49B96
SMRiQ49B96
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini27 – 69CHCHPROSITE-ProRule annotationAdd BLAST43

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi30 – 40Cx9C motif 1PROSITE-ProRule annotationAdd BLAST11
Motifi51 – 61Cx9C motif 2PROSITE-ProRule annotationAdd BLAST11

Sequence similaritiesi

Belongs to the COX19 family.Curated

Phylogenomic databases

eggNOGiKOG3477 Eukaryota
ENOG41121W4 LUCA
GeneTreeiENSGT00390000016895
HOGENOMiHOG000208239
HOVERGENiHBG081305
InParanoidiQ49B96
KOiK18183
OMAiDYLDCRM
OrthoDBiEOG091G10VA
PhylomeDBiQ49B96
TreeFamiTF321525

Family and domain databases

InterProiView protein in InterPro
IPR010625 CHCH
IPR009069 Cys_alpha_HP_mot_SF
PfamiView protein in Pfam
PF06747 CHCH, 1 hit
SUPFAMiSSF47072 SSF47072, 1 hit
PROSITEiView protein in PROSITE
PS51808 CHCH, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q49B96-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MSTAMNFGTK SFQPRPPDKG SFPLDHLGEC KSFKEKFMKC LHNNNFENAL
60 70 80 90
CRKESKEYLE CRMERKLMLQ EPLEKLGFGD LTSGKSEAKK
Length:90
Mass (Da):10,394
Last modified:September 13, 2005 - v1
Checksum:i238A37E8A70EFA09
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY957566 mRNA Translation: AAY35062.1
BC070383 mRNA Translation: AAH70383.1
BC103632 mRNA Translation: AAI03633.1
BC110420 mRNA Translation: AAI10421.1
CCDSiCCDS34582.1
RefSeqiNP_001026788.1, NM_001031617.2
UniGeneiHs.121593

Genome annotation databases

EnsembliENST00000344111; ENSP00000342015; ENSG00000240230
GeneIDi90639
KEGGihsa:90639
UCSCiuc003sjp.2 human

Similar proteinsi

Entry informationi

Entry nameiCOX19_HUMAN
AccessioniPrimary (citable) accession number: Q49B96
Secondary accession number(s): A4FTX0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: September 13, 2005
Last modified: March 28, 2018
This is version 101 of the entry and version 1 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, Reference proteome