Q9Y6N1 (COX11_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Cytochrome c oxidase assembly protein COX11, mitochondrial

Gene names

Name:

COX11

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	276 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	Exerts its effect at some terminal stage of cytochrome c oxidase synthesis, probably by being involved in the insertion of the copper B into subunit I By similarity.
Subunit structure	Interacts with CNNM4/ACDP4. Ref.7
Subcellular location	Mitochondrion inner membrane; Single-pass membrane protein; Intermembrane side Ref.1.
Tissue specificity	Ubiquitous. Ref.1
Sequence similarities	Belongs to the COX11/CtaG family.

Ontologies

Keywords
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Transit peptide Transmembrane Transmembrane helix
Ligand	Copper
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	respiratory chain complex IV assembly Traceable author statement Ref.1. Source: ProtInc respiratory gaseous exchange Traceable author statement Ref.1. Source: ProtInc
Cellular_component	integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW mitochondrial inner membrane Inferred from electronic annotation. Source: UniProtKB-SubCell mitochondrion Traceable author statement Ref.1. Source: ProtInc
Molecular_function	copper ion binding Inferred from electronic annotation. Source: InterPro cytochrome-c oxidase activity Traceable author statement Ref.1. Source: ProtInc electron carrier activity Traceable author statement Ref.1. Source: UniProtKB
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: Q9Y6N1-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

Isoform 2 (identifier: Q9Y6N1-2)

The sequence of this isoform differs from the canonical sequence as follows:
     217-276: CFCFEEQRLNPQEEVDMPVFFYIDPEFAEDPRMIKVDLITLSYTFFEAKEGHKLPVPGYN → ASKLHRVYVLESWHL

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – ?		Mitochondrion Potential
	Chain	? – 276		Cytochrome c oxidase assembly protein COX11, mitochondrial		PRO_0000006080
Regions
	Topological domain	? – 95		Mitochondrial matrix Potential
	Transmembrane	96 – 114	19	Helical; Potential
	Topological domain	115 – 276	162	Mitochondrial intermembrane Potential
Natural variations
	Alternative sequence	217 – 276	60	CFCFE…VPGYN → ASKLHRVYVLESWHL in isoform 2.		VSP_046360
	Natural variant	74	1	P → L. Corresponds to variant rs34080917 [ dbSNP | Ensembl ].		VAR_048831
Experimental info
	Sequence conflict	187	1	A → V in AAH05895. Ref.5
Isoform 2:
	Sequence conflict	223	1	V → G in BI600359. Ref.4

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified March 1, 2005. Version 3. Checksum: 1F2BD22E1E7DD52D	FASTA	276	31,430
	10 20 30 40 50 60 MGGLWRPGWR CVPFCGWRWI HPGSPTRAAE RVEPFLRPEW SGTGGAERGL RWLGTWKRCS 70 80 90 100 110 120 LRARHPALQP PRRPKSSNPF TRAQEEERRR QNKTTLTYVA AVAVGMLGAS YAAVPLYRLY 130 140 150 160 170 180 CQTTGLGGSA VAGHASDKIE NMVPVKDRII KISFNADVHA SLQWNFRPQQ TEIYVVPGET 190 200 210 220 230 240 ALAFYRAKNP TDKPVIGIST YNIVPFEAGQ YFNKIQCFCF EEQRLNPQEE VDMPVFFYID 250 260 270 PEFAEDPRMI KVDLITLSYT FFEAKEGHKL PVPGYN
	Isoform 2 [UniParc]. Checksum: 3A8BC28441A3D78B	FASTA	231	26,133
	10 20 30 40 50 60 MGGLWRPGWR CVPFCGWRWI HPGSPTRAAE RVEPFLRPEW SGTGGAERGL RWLGTWKRCS 70 80 90 100 110 120 LRARHPALQP PRRPKSSNPF TRAQEEERRR QNKTTLTYVA AVAVGMLGAS YAAVPLYRLY 130 140 150 160 170 180 CQTTGLGGSA VAGHASDKIE NMVPVKDRII KISFNADVHA SLQWNFRPQQ TEIYVVPGET 190 200 210 220 230 ALAFYRAKNP TDKPVIGIST YNIVPFEAGQ YFNKIQASKL HRVYVLESWH L

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain." Petruzzella V., Tiranti V., Fernandez P., Ianna P., Carrozzo R., Zeviani M. Genomics 54:494-504(1998) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), SUBCELLULAR LOCATION, TISSUE SPECIFICITY.
[2]	"Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)." Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B. Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
[3]	"DNA sequence of human chromosome 17 and analysis of rearrangement in the human lineage." Zody M.C., Garber M., Adams D.J., Sharpe T., Harrow J., Lupski J.R., Nicholson C., Searle S.M., Wilming L., Young S.K., Abouelleil A., Allen N.R., Bi W., Bloom T., Borowsky M.L., Bugalter B.E., Butler J., Chang J.L. , Chen C.-K., Cook A., Corum B., Cuomo C.A., de Jong P.J., DeCaprio D., Dewar K., FitzGerald M., Gilbert J., Gibson R., Gnerre S., Goldstein S., Grafham D.V., Grocock R., Hafez N., Hagopian D.S., Hart E., Norman C.H., Humphray S., Jaffe D.B., Jones M., Kamal M., Khodiyar V.K., LaButti K., Laird G., Lehoczky J., Liu X., Lokyitsang T., Loveland J., Lui A., Macdonald P., Major J.E., Matthews L., Mauceli E., McCarroll S.A., Mihalev A.H., Mudge J., Nguyen C., Nicol R., O'Leary S.B., Osoegawa K., Schwartz D.C., Shaw-Smith C., Stankiewicz P., Steward C., Swarbreck D., Venkataraman V., Whittaker C.A., Yang X., Zimmer A.R., Bradley A., Hubbard T., Birren B.W., Rogers J., Lander E.S., Nusbaum C. Nature 440:1045-1049(2006) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2). Tissue: Brain and Hypothalamus.
[6]	"Large-scale concatenation cDNA sequencing." Yu W., Andersson B., Worley K.C., Muzny D.M., Ding Y., Liu W., Ricafrente J.Y., Wentland M.A., Lennon G., Gibbs R.A. Genome Res. 7:353-358(1997) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 125-276 (ISOFORM 1). Tissue: Brain.
[7]	"Physical interaction and functional coupling between ACDP4 and the intracellular ion chaperone COX11, an implication of the role of ACDP4 in essential metal ion transport and homeostasis." Guo D., Ling J., Wang M.-H., She J.-X., Gu J., Wang C.-Y. Mol. Pain 1:15-15(2005) [PubMed] [Europe PMC] [Abstract] Cited for: INTERACTION WITH CNNM4.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AF044321 mRNA. Translation: AAD08645.1. CR541837 mRNA. Translation: CAG46636.1. AC007485 Genomic DNA. No translation available. AC090824 Genomic DNA. No translation available. CH471109 Genomic DNA. Translation: EAW94548.1. CH471109 Genomic DNA. Translation: EAW94550.1. BC005895 mRNA. Translation: AAH05895.1. BI600359 mRNA. No translation available. U79270 mRNA. Translation: AAB50214.1.
IPI	IPI00295394.
RefSeq	NP_004366.1. NM_004375.3.
UniGene	Hs.591171.
3D structure databases
ProteinModelPortal	Q9Y6N1.
SMR	Q9Y6N1. Positions 145-262.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q9Y6N1. 2 interactions.
STRING	9606.ENSP00000299335.
PTM databases
PhosphoSite	Q9Y6N1.
Polymorphism databases
DMDM	60416378.
Proteomic databases
PaxDb	Q9Y6N1.
PRIDE	Q9Y6N1.
Protocols and materials databases
DNASU	1353.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000299335; ENSP00000299335; ENSG00000166260. ENST00000572558; ENSP00000459302; ENSG00000166260. ENST00000576370; ENSP00000459901; ENSG00000166260.
GeneID	1353.
KEGG	hsa:1353.
UCSC	uc010wng.1. human.
Organism-specific databases
CTD	1353.
GeneCards	GC17M053029.
HGNC	HGNC:2261. COX11.
HPA	HPA044020.
MIM	603648. gene.
neXtProt	NX_Q9Y6N1.
PharmGKB	PA26777.
GenAtlas	Search...
Phylogenomic databases
eggNOG	COG3175.
HOGENOM	HOG000264965.
HOVERGEN	HBG051085.
InParanoid	Q9Y6N1.
KO	K02258.
OMA	CEITGIN.
OrthoDB	EOG4MGS7W.
PhylomeDB	Q9Y6N1.
Gene expression databases
ArrayExpress	Q9Y6N1.
Bgee	Q9Y6N1.
CleanEx	HS_COX11.
Genevestigator	Q9Y6N1.
GermOnline	ENSG00000166260. Homo sapiens.
Family and domain databases
Gene3D	2.60.370.10. 1 hit.
InterPro	IPR023471. CtaG/Cox11_dom. IPR007533. Cyt_c_oxidase_assmbl_CtaG. [Graphical view]
PANTHER	PTHR21320. PTHR21320. 1 hit.
Pfam	PF04442. CtaG_Cox11. 1 hit. [Graphical view]
SUPFAM	SSF110111. CtaG_Cox11. 1 hit.
ProtoNet	Search...
Other
GenomeRNAi	1353.
NextBio	5483.
SOURCE	Search...

Entry information

Entry name

COX11_HUMAN

Accession

Primary (citable) accession number: Q9Y6N1
Secondary accession number(s): D3DTY5 , I3L220, Q6FHB7, Q9BRX0, Q9UME8

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 30, 2000
Last sequence update:	March 1, 2005
Last modified:	May 1, 2013
This is version 109 of the entry and version 3 of the sequence. [Complete history]

Entry status

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.