COX6A1

Functionⁱ

This protein is one of the nuclear-coded polypeptide chains of cytochrome c oxidase, the terminal oxidase in mitochondrial electron transport.

GO - Molecular functionⁱ

cytochrome-c oxidase activity Source: InterPro
enzyme regulator activity
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

aerobic respiration
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
generation of precursor metabolites and energy
Source: ProtInc
Traceable author statementⁱ
- 2549515
mitochondrial electron transport, cytochrome c to oxygen
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635

View the complete GO annotation on QuickGO ...

Enzyme and pathway databases

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

Reactomeⁱ

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

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Cytochrome c oxidase subunit 6A1, mitochondrial Alternative name(s): Cytochrome c oxidase polypeptide VIa-liver Cytochrome c oxidase subunit VIA-liver Short name: COX VIa-L
Gene namesⁱ	Name:COX6A1 Synonyms:COX6AL
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 12

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000111775.2
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:2277 COX6A1
MIMⁱ	602072 gene
neXtProtⁱ	NX_P12074

Keywords - Cellular componentⁱ

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Charcot-Marie-Tooth disease, recessive, intermediate type, D (CMTRID)1 Publication

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

Disease descriptionA form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Recessive intermediate forms of Charcot-Marie-Tooth disease are characterized by clinical and pathologic features intermediate between demyelinating and axonal peripheral neuropathies, and motor median nerve conduction velocities ranging from 25 to 45 m/sec.

Keywords - Diseaseⁱ

Charcot-Marie-Tooth disease, Neurodegeneration, Neuropathy

Organism-specific databases

DisGeNET More...DisGeNETⁱ	1337
MalaCards human disease database More...MalaCardsⁱ	COX6A1
MIMⁱ	616039 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000111775
Orphanetⁱ	435998 Autosomal recessive intermediate Charcot-Marie-Tooth disease type D
PharmGKBⁱ	PA26794

Polymorphism and mutation databases

BioMutaⁱ	COX6A1
DMDMⁱ	6166030

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Transit peptideⁱ	1 – 24	Mitochondrion1 PublicationAdd BLAST		24
ChainⁱPRO_0000006119	25 – 109	Cytochrome c oxidase subunit 6A1, mitochondrialAdd BLAST		85

Proteomic databases

EPDⁱ	P12074
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P12074
PaxDbⁱ	P12074
PeptideAtlas More...PeptideAtlasⁱ	P12074
PRIDEⁱ	P12074
ProteomicsDBⁱ	52823
TopDownProteomicsⁱ	P12074

2D gel databases

SWISS-2DPAGEⁱ	P12074

SWISS-2DPAGEⁱ

P12074

PTM databases

iPTMnetⁱ	P12074
PhosphoSitePlusⁱ	P12074

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000111775 Expressed in 92 organ(s), highest expression level in prefrontal cortex
CleanExⁱ	HS_COX6A1
ExpressionAtlasⁱ	P12074 baseline and differential
Genevisibleⁱ	P12074 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA062394

HPAⁱ

HPA062394

Interactionⁱ

Protein-protein interaction databases

BioGridⁱ	107730, 16 interactors
IntActⁱ	P12074, 14 interactors
STRINGⁱ	9606.ENSP00000229379

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	P12074
SMRⁱ	P12074
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the cytochrome c oxidase subunit 6A family.Curated

Keywords - Domainⁱ

Transit peptide

Phylogenomic databases

eggNOGⁱ	KOG3469 Eukaryota ENOG411253X LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000002756
HOGENOMⁱ	HOG000193965
HOVERGENⁱ	HBG060482
InParanoidⁱ	P12074
KEGG Orthology (KO) More...KOⁱ	K02266
OMAⁱ	GYEGHDE
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G0C5F
PhylomeDBⁱ	P12074
TreeFamⁱ	TF105064

Family and domain databases

Conserved Domains Database More...CDDⁱ	cd00925 Cyt_c_Oxidase_VIa, 1 hit
Gene3Dⁱ	4.10.95.10, 1 hit
InterProⁱ	View protein in InterPro IPR001349 Cyt_c_oxidase_su6a IPR018507 Cyt_c_oxidase_su6a_CS IPR036418 Cyt_c_oxidase_su6a_sf
PANTHERⁱ	PTHR11504 PTHR11504, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF02046 COX6A, 1 hit
PIRSFⁱ	PIRSF000277 COX6A1, 1 hit
ProDomⁱ	View protein in ProDom or Entries sharing at least one domain PD006036 Cyt_c_oxidase_su6a, 1 hit
SUPFAMⁱ	SSF81411 SSF81411, 1 hit
PROSITEⁱ	View protein in PROSITE PS01329 COX6A, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

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

P12074-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MAVVGVSSVS RLLGRSRPQL GRPMSSGAHG EEGSARMWKT LTFFVALPGV 
        60         70         80         90        100
AVSMLNVYLK SHHGEHERPE FIAYPHLRIR TKPFPWGDGN HTLFHNPHVN 

PLPTGYEDE

Length:109

Mass (Da):12,155

Last modified:July 15, 1999 - v4

Checksum:ⁱ0040F47CECE767B1

GO

Sequence cautionⁱ

The sequence CAA33392 differs from that shown. Reason: Frameshift at position 17.Curated

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK312009 mRNA Translation: BAG34947.1 AL021546 Genomic DNA No translation available. BC007723 mRNA Translation: AAH07723.1 BC070186 mRNA Translation: AAH70186.1 BC107861 mRNA Translation: AAI07862.1 X15341 mRNA Translation: CAA33392.1 Frameshift.
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS9197.1
NCBI Reference Sequences More...RefSeqⁱ	NP_004364.2, NM_004373.3
UniGeneⁱ	Hs.369624

Genome annotation databases

Ensemblⁱ	ENST00000229379; ENSP00000229379; ENSG00000111775
GeneIDⁱ	1337
KEGGⁱ	hsa:1337
UCSC genome browser More...UCSCⁱ	uc001tyf.2 human

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P12074	Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		152	UniRef100_P12074
Cytochrome c oxidase subunit 6A, mitochondrial		PANTR		109
Cytochrome c oxidase subunit 6A, mitochondrial		PANPA		109
Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		109
Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		86

Protein	Similar proteins		Species	Score	Length	Source
P12074	Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		152	UniRef90_P12074
Cytochrome c oxidase subunit 6A, mitochondrial		PANTR		109
Cytochrome c oxidase subunit 6A, mitochondrial		PANPA		109
Cytochrome c oxidase subunit 6A, mitochondrial		PAPAN		109
Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		109
+2

Protein	Similar proteins		Species	Score	Length	Source
P12074	Cytochrome c oxidase subunit 6A1, mitochondrial		RABIT		109	UniRef50_P12074
Cytochrome c oxidase subunit 6A, mitochondrial		HUMAN		152
Cytochrome c oxidase subunit 6A, mitochondrial		PANTR		109
Cytochrome c oxidase subunit 6A, mitochondrial		PANPA		109
Cytochrome c oxidase subunit 6A, mitochondrial		PAPAN		109
+39

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK312009 mRNA Translation: BAG34947.1 AL021546 Genomic DNA No translation available. BC007723 mRNA Translation: AAH07723.1 BC070186 mRNA Translation: AAH70186.1 BC107861 mRNA Translation: AAI07862.1 X15341 mRNA Translation: CAA33392.1 Frameshift.
CCDSⁱ	CCDS9197.1
RefSeqⁱ	NP_004364.2, NM_004373.3
UniGeneⁱ	Hs.369624

3D structure databases

ProteinModelPortalⁱ	P12074
SMRⁱ	P12074
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	107730, 16 interactors
IntActⁱ	P12074, 14 interactors
STRINGⁱ	9606.ENSP00000229379

PTM databases

iPTMnetⁱ	P12074
PhosphoSitePlusⁱ	P12074

Polymorphism and mutation databases

BioMutaⁱ	COX6A1
DMDMⁱ	6166030

2D gel databases

SWISS-2DPAGEⁱ	P12074

SWISS-2DPAGEⁱ

P12074

Proteomic databases

EPDⁱ	P12074
MaxQBⁱ	P12074
PaxDbⁱ	P12074
PeptideAtlasⁱ	P12074
PRIDEⁱ	P12074
ProteomicsDBⁱ	52823
TopDownProteomicsⁱ	P12074

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	1337
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000229379; ENSP00000229379; ENSG00000111775
GeneIDⁱ	1337
KEGGⁱ	hsa:1337
UCSCⁱ	uc001tyf.2 human

Organism-specific databases

CTDⁱ	1337
DisGeNETⁱ	1337
EuPathDBⁱ	HostDB:ENSG00000111775.2
GeneCardsⁱ	COX6A1
HGNCⁱ	HGNC:2277 COX6A1
HPAⁱ	HPA062394
MalaCardsⁱ	COX6A1
MIMⁱ	602072 gene 616039 phenotype
neXtProtⁱ	NX_P12074
OpenTargetsⁱ	ENSG00000111775
Orphanetⁱ	435998 Autosomal recessive intermediate Charcot-Marie-Tooth disease type D
PharmGKBⁱ	PA26794
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG3469 Eukaryota ENOG411253X LUCA
GeneTreeⁱ	ENSGT00390000002756
HOGENOMⁱ	HOG000193965
HOVERGENⁱ	HBG060482
InParanoidⁱ	P12074
KOⁱ	K02266
OMAⁱ	GYEGHDE
OrthoDBⁱ	EOG091G0C5F
PhylomeDBⁱ	P12074
TreeFamⁱ	TF105064

Enzyme and pathway databases

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

Reactomeⁱ

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

Miscellaneous databases

ChiTaRSⁱ	COX6A1 human
GeneWikiⁱ	COX6A1
GenomeRNAiⁱ	1337
Protein Ontology More...PROⁱ	PR:P12074
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000111775 Expressed in 92 organ(s), highest expression level in prefrontal cortex
CleanExⁱ	HS_COX6A1
ExpressionAtlasⁱ	P12074 baseline and differential
Genevisibleⁱ	P12074 HS

Family and domain databases

CDDⁱ	cd00925 Cyt_c_Oxidase_VIa, 1 hit
Gene3Dⁱ	4.10.95.10, 1 hit
InterProⁱ	View protein in InterPro IPR001349 Cyt_c_oxidase_su6a IPR018507 Cyt_c_oxidase_su6a_CS IPR036418 Cyt_c_oxidase_su6a_sf
PANTHERⁱ	PTHR11504 PTHR11504, 1 hit
Pfamⁱ	View protein in Pfam PF02046 COX6A, 1 hit
PIRSFⁱ	PIRSF000277 COX6A1, 1 hit
ProDomⁱ	View protein in ProDom or Entries sharing at least one domain PD006036 Cyt_c_oxidase_su6a, 1 hit
SUPFAMⁱ	SSF81411 SSF81411, 1 hit
PROSITEⁱ	View protein in PROSITE PS01329 COX6A, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	CX6A1_HUMAN
Accessionⁱ	P12074Primary (citable) accession number: P12074 Secondary accession number(s): B2R500, O43714, Q32Q37
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	October 1, 1989
	Last sequence update:	July 15, 1999
	Last modified:	November 7, 2018
	This is version 176 of the entry and version 4 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, Direct protein sequencing, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
Human chromosome 12
Human chromosome 12: entries, gene names and cross-references to MIM

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UniProtKB - P12074 (CX6A1_HUMAN)

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Cytochrome c oxidase subunit 6A1, mitochondrial

Select a section on the left to see content.

<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 - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<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

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

Keywords - Diseasei

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

Proteomic databases

2D gel 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

<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

Keywords - Domaini

Phylogenomic databases

Family and domain databases

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

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

2D gel databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway 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

Keywords - Technical termi

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Sequence cautionⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ