COA5

Functionⁱ

Involved in an early step of the mitochondrial complex IV assembly process.1 Publication

GO - Biological processⁱ

mitochondrial respiratory chain complex IV assembly Source: GO_Central

View the complete GO annotation on QuickGO ...

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Cytochrome c oxidase assembly factor 5
Gene namesⁱ	Name:COA5 Synonyms:C2orf64
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 2

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000183513.8.
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:33848. COA5.
MIMⁱ	613920. gene.
neXtProtⁱ	NX_Q86WW8.

Subcellular locationⁱ

GO - Cellular component

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 3 (CEMCOX3)1 Publication

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionAn infantile disorder with a fatal course in the first weeks of life, characterized by hypertrophic cardiomyopathy and mitochondrial complex IV deficiency. Postmortem microscopic investigations show accumulation of lipid droplets in cardiomyocytes and mitochondrial proliferation.

Keywords - Diseaseⁱ

Disease mutation, Primary mitochondrial disease

Organism-specific databases

DisGeNET More...DisGeNETⁱ	493753.
MalaCards human disease database More...MalaCardsⁱ	COA5.
MIMⁱ	616500. phenotype.
Open Targets More...OpenTargetsⁱ	ENSG00000183513.
Orphanetⁱ	254905. Isolated cytochrome C oxidase deficiency. 70474. Leigh syndrome with cardiomyopathy.
PharmGKBⁱ	PA162379390.

Polymorphism and mutation databases

BioMutaⁱ	COA5.
DMDMⁱ	74727729.

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000325876	1 – 74	Cytochrome c oxidase assembly factor 5Add BLAST		74

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Disulfide bondⁱ	30 ↔ 57	PROSITE-ProRule annotation
Modified residueⁱ	37	PhosphoserineCombined sources	1
Disulfide bondⁱ	41 ↔ 47	PROSITE-ProRule annotation

Keywords - PTMⁱ

Disulfide bond, Phosphoprotein

Proteomic databases

EPDⁱ	Q86WW8.
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q86WW8.
PaxDbⁱ	Q86WW8.
PeptideAtlas More...PeptideAtlasⁱ	Q86WW8.
PRIDEⁱ	Q86WW8.

PTM databases

iPTMnetⁱ	Q86WW8.
PhosphoSitePlusⁱ	Q86WW8.

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000183513.
CleanExⁱ	HS_C2orf64.
ExpressionAtlasⁱ	Q86WW8. baseline and differential.
Genevisibleⁱ	Q86WW8. HS.

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA057768.

HPAⁱ

HPA057768.

Interactionⁱ

Binary interactionsⁱ

With	Entry	#Exp.	IntAct	Notes
KRT31	Q15323	3	EBI-5458774,EBI-948001

With

Entry

#Exp.

IntAct

Notes

KRT31

Q15323

3

EBI-5458774,EBI-948001

Protein-protein interaction databases

BioGridⁱ	138902. 3 interactors.
IntActⁱ	Q86WW8. 4 interactors.
Molecular INTeraction database More...MINTⁱ	Q86WW8.
STRINGⁱ	9606.ENSP00000330730.

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q86WW8.
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Domains and Repeats

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Domainⁱ	27 – 65	CHCHPROSITE-ProRule annotationAdd BLAST		39

Motif

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Motifⁱ	30 – 41	Cx10C motifPROSITE-ProRule annotationAdd BLAST		12
Motifⁱ	47 – 57	Cx9C motifPROSITE-ProRule annotationAdd BLAST		11

Sequence similaritiesⁱ

Belongs to the PET191 family.Curated

Phylogenomic databases

eggNOGⁱ	KOG4114. Eukaryota. ENOG4111YIY. LUCA.
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000005548.
HOVERGENⁱ	HBG107093.
InParanoidⁱ	Q86WW8.
KEGG Orthology (KO) More...KOⁱ	K18178.
OMAⁱ	DMRSRFR.
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0ZD7.
PhylomeDBⁱ	Q86WW8.
TreeFamⁱ	TF313953.

Family and domain databases

InterProⁱ	View protein in InterPro IPR018793. Cyt_c_oxidase_assmbl_Pet191.
PANTHERⁱ	PTHR28627. PTHR28627. 1 hit.
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF10203. Pet191_N. 1 hit.
PROSITEⁱ	View protein in PROSITE PS51808. CHCH. 1 hit.

Sequenceⁱ

Sequence statusⁱ: Complete.

Q86WW8-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MPKYYEDKPQ GGACAGLKED LGACLLQSDC VVQEGKSPRQ CLKEGYCNSL 
        60         70 
KYAFFECKRS VLDNRARFRG RKGY

Length:74

Mass (Da):8,376

Last modified:June 1, 2003 - v1

Checksum:ⁱCE4D3FF94332B2A9

GO

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_065499	53	A → P in CEMCOX3. 1 PublicationCorresponds to variant dbSNP:rs387907099Ensembl.		1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AC010134 Genomic DNA. Translation: AAX93231.1. CH471127 Genomic DNA. Translation: EAX01903.1. BC047722 mRNA. Translation: AAH47722.1.
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS33257.1.
NCBI Reference Sequences More...RefSeqⁱ	NP_001008216.1. NM_001008215.2.
UniGeneⁱ	Hs.596537.

Genome annotation databases

Ensemblⁱ	ENST00000328709; ENSP00000330730; ENSG00000183513.
GeneIDⁱ	493753.
KEGGⁱ	hsa:493753.
UCSC genome browser More...UCSCⁱ	uc002syz.4. human.

Protein	Similar proteins	Organisms	Length	Cluster ID	Cluster name	Size
Q86WW8	H2QIE5 G3QT78	Pan troglodytes (Chimpanzee) Gorilla gorilla gorilla (Western lowland gorilla)	74	UniRef100_Q86WW8	Cluster: Cytochrome c oxidase assembly factor 5	3

Protein	Similar proteins	Organisms	Length	Cluster ID	Cluster name	Size
Q86WW8	H2QIE5 G3QT78 Q5RFJ0 A0A2J8RJC8 A0A0D9RXD4 A0A2K6JR12 A0A2K5KZ23 A0A2K6NS79 A0A096MX89 A0A2K5H6P5 +2	Pan troglodytes (Chimpanzee) Gorilla gorilla gorilla (Western lowland gorilla) Pongo abelii (Sumatran orangutan) (Pongo pygmaeus abelii) Chlorocebus sabaeus (Green monkey) (Cercopithecus sabaeus) Rhinopithecus bieti (Black snub-nosed monkey) (Pygathrix bieti) Cercocebus atys (Sooty mangabey) (Cercocebus torquatus atys) Rhinopithecus roxellana (Golden snub-nosed monkey) (Pygathrix roxellana) Papio anubis (Olive baboon) Colobus angolensis palliatus (Peters' Angolan colobus) Nomascus leucogenys (Northern white-cheeked gibbon) (Hylobates leucogenys)	74	UniRef90_Q86WW8	Cluster: Cytochrome c oxidase assembly factor 5	13

Protein	Similar proteins	Organisms	Length	Cluster ID	Cluster name	Size
Q86WW8	H2QIE5 G3QT78 Q5RFJ0 A0A2J8RJC8 A0A2I3GQ73 UPI00062A6961 A0A0D9RXD4 A0A2K6JR12 A0A2K5KZ23 A0A2K6NS79 +37	Pan troglodytes (Chimpanzee) Gorilla gorilla gorilla (Western lowland gorilla) Pongo abelii (Sumatran orangutan) (Pongo pygmaeus abelii) Nomascus leucogenys (Northern white-cheeked gibbon) (Hylobates leucogenys) Chlorocebus sabaeus (Green monkey) (Cercopithecus sabaeus) Rhinopithecus bieti (Black snub-nosed monkey) (Pygathrix bieti) Cercocebus atys (Sooty mangabey) (Cercocebus torquatus atys) Rhinopithecus roxellana (Golden snub-nosed monkey) (Pygathrix roxellana) Papio anubis (Olive baboon) Colobus angolensis palliatus (Peters' Angolan colobus) And more	74	UniRef50_Q86WW8	Cluster: Cytochrome c oxidase assembly factor 5	48

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AC010134 Genomic DNA. Translation: AAX93231.1. CH471127 Genomic DNA. Translation: EAX01903.1. BC047722 mRNA. Translation: AAH47722.1.
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS33257.1.
NCBI Reference Sequences More...RefSeqⁱ	NP_001008216.1. NM_001008215.2.
UniGeneⁱ	Hs.596537.

3D structure databases

ProteinModelPortalⁱ	Q86WW8.
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	138902. 3 interactors.
IntActⁱ	Q86WW8. 4 interactors.
Molecular INTeraction database More...MINTⁱ	Q86WW8.
STRINGⁱ	9606.ENSP00000330730.

PTM databases

iPTMnetⁱ	Q86WW8.
PhosphoSitePlusⁱ	Q86WW8.

Polymorphism and mutation databases

BioMutaⁱ	COA5.
DMDMⁱ	74727729.

Proteomic databases

EPDⁱ	Q86WW8.
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q86WW8.
PaxDbⁱ	Q86WW8.
PeptideAtlas More...PeptideAtlasⁱ	Q86WW8.
PRIDEⁱ	Q86WW8.

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	493753.
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000328709; ENSP00000330730; ENSG00000183513.
GeneIDⁱ	493753.
KEGGⁱ	hsa:493753.
UCSC genome browser More...UCSCⁱ	uc002syz.4. human.

Organism-specific databases

CTDⁱ	493753.
DisGeNET More...DisGeNETⁱ	493753.
EuPathDBⁱ	HostDB:ENSG00000183513.8.
GeneCardsⁱ	COA5.
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:33848. COA5.
Human Protein Atlas More...HPAⁱ	HPA057768.
MalaCards human disease database More...MalaCardsⁱ	COA5.
MIMⁱ	613920. gene. 616500. phenotype.
neXtProtⁱ	NX_Q86WW8.
Open Targets More...OpenTargetsⁱ	ENSG00000183513.
Orphanetⁱ	254905. Isolated cytochrome C oxidase deficiency. 70474. Leigh syndrome with cardiomyopathy.
PharmGKBⁱ	PA162379390.
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG4114. Eukaryota. ENOG4111YIY. LUCA.
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000005548.
HOVERGENⁱ	HBG107093.
InParanoidⁱ	Q86WW8.
KEGG Orthology (KO) More...KOⁱ	K18178.
OMAⁱ	DMRSRFR.
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0ZD7.
PhylomeDBⁱ	Q86WW8.
TreeFamⁱ	TF313953.

Miscellaneous databases

GenomeRNAiⁱ	493753.
Protein Ontology More...PROⁱ	PR:Q86WW8.
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000183513.
CleanExⁱ	HS_C2orf64.
ExpressionAtlasⁱ	Q86WW8. baseline and differential.
Genevisibleⁱ	Q86WW8. HS.

Family and domain databases

InterProⁱ	View protein in InterPro IPR018793. Cyt_c_oxidase_assmbl_Pet191.
PANTHERⁱ	PTHR28627. PTHR28627. 1 hit.
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF10203. Pet191_N. 1 hit.
PROSITEⁱ	View protein in PROSITE PS51808. CHCH. 1 hit.
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	COA5_HUMAN
Accessionⁱ	Q86WW8Primary (citable) accession number: Q86WW8
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	March 18, 2008
	Last sequence update:	June 1, 2003
	Last modified:	February 28, 2018
	This is version 105 of the entry and version 1 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

Complete proteome, Reference proteome

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UniParc

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UniRef

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Annotation systems

Supporting data

UniProtKB - Q86WW8 (COA5_HUMAN)

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Cytochrome c oxidase assembly factor 5

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<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Biological processi

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Motif

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

Natural variant

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

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Functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ