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Protein

Cytochrome c oxidase assembly protein COX11, mitochondrial

Gene

COX11

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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

GO - Molecular functioni

  1. copper ion binding Source: InterPro
  2. cytochrome-c oxidase activity Source: ProtInc
  3. electron carrier activity Source: UniProtKB

GO - Biological processi

  1. hydrogen ion transmembrane transport Source: GOC
  2. negative regulation of glucokinase activity Source: Ensembl
  3. respiratory chain complex IV assembly Source: ProtInc
  4. respiratory gaseous exchange Source: ProtInc
Complete GO annotation...

Keywords - Ligandi

Copper

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.
REACT_267716. Orphan transporters.

Names & Taxonomyi

Protein namesi
Recommended name:
Cytochrome c oxidase assembly protein COX11, mitochondrial
Gene namesi
Name:COX11
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 17

Organism-specific databases

HGNCiHGNC:2261. COX11.

Subcellular locationi

Mitochondrion inner membrane 1 Publication; Single-pass membrane protein 1 Publication; Intermembrane side 1 Publication

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini? – 95Mitochondrial matrixSequence Analysis
Transmembranei96 – 11419HelicalSequence AnalysisAdd
BLAST
Topological domaini115 – 276162Mitochondrial intermembraneSequence AnalysisAdd
BLAST

GO - Cellular componenti

  1. integral component of membrane Source: UniProtKB-KW
  2. mitochondrial inner membrane Source: UniProtKB-SubCell
  3. mitochondrion Source: ProtInc
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA26777.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini? – 276Cytochrome c oxidase assembly protein COX11, mitochondrialPRO_0000006080
Transit peptidei1 – ?MitochondrionSequence Analysis

Proteomic databases

MaxQBiQ9Y6N1.
PaxDbiQ9Y6N1.
PRIDEiQ9Y6N1.

PTM databases

PhosphoSiteiQ9Y6N1.

Expressioni

Tissue specificityi

Ubiquitous.1 Publication

Gene expression databases

BgeeiQ9Y6N1.
CleanExiHS_COX11.
ExpressionAtlasiQ9Y6N1. baseline and differential.
GenevestigatoriQ9Y6N1.

Organism-specific databases

HPAiHPA044020.

Interactioni

Subunit structurei

Interacts with CNNM4/ACDP4.1 Publication

Protein-protein interaction databases

BioGridi107746. 3 interactions.
IntActiQ9Y6N1. 3 interactions.
STRINGi9606.ENSP00000299335.

Structurei

3D structure databases

ProteinModelPortaliQ9Y6N1.
SMRiQ9Y6N1. Positions 145-262.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the COX11/CtaG family.Curated

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG3175.
GeneTreeiENSGT00390000007512.
HOGENOMiHOG000264965.
HOVERGENiHBG051085.
InParanoidiQ9Y6N1.
KOiK02258.
OMAiRMARVDT.
OrthoDBiEOG7JMGFB.
PhylomeDBiQ9Y6N1.
TreeFamiTF105072.

Family and domain databases

Gene3Di2.60.370.10. 1 hit.
HAMAPiMF_00155. CtaG.
InterProiIPR023471. CtaG/Cox11_dom.
IPR007533. Cyt_c_oxidase_assmbl_CtaG.
[Graphical view]
PANTHERiPTHR21320. PTHR21320. 1 hit.
PfamiPF04442. CtaG_Cox11. 1 hit.
[Graphical view]
SUPFAMiSSF110111. SSF110111. 1 hit.

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. Align

Isoform 1 (identifier: Q9Y6N1-1) [UniParc]FASTAAdd to Basket

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.

« Hide

        10         20         30         40         50
MGGLWRPGWR CVPFCGWRWI HPGSPTRAAE RVEPFLRPEW SGTGGAERGL
60 70 80 90 100
RWLGTWKRCS LRARHPALQP PRRPKSSNPF TRAQEEERRR QNKTTLTYVA
110 120 130 140 150
AVAVGMLGAS YAAVPLYRLY CQTTGLGGSA VAGHASDKIE NMVPVKDRII
160 170 180 190 200
KISFNADVHA SLQWNFRPQQ TEIYVVPGET ALAFYRAKNP TDKPVIGIST
210 220 230 240 250
YNIVPFEAGQ YFNKIQCFCF EEQRLNPQEE VDMPVFFYID PEFAEDPRMI
260 270
KVDLITLSYT FFEAKEGHKL PVPGYN
Length:276
Mass (Da):31,430
Last modified:March 1, 2005 - v3
Checksum:i1F2BD22E1E7DD52D
GO
Isoform 2 (identifier: Q9Y6N1-2) [UniParc]FASTAAdd to Basket

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

Note: No experimental confirmation available.Curated

Show »
Length:231
Mass (Da):26,133
Checksum:i3A8BC28441A3D78B
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti187 – 1871A → V in AAH05895. (PubMed:15489334)Curated
Isoform 2 (identifier: Q9Y6N1-2)
Sequence conflicti223 – 2231V → G in BI600359. 1 PublicationCurated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti74 – 741P → L.
Corresponds to variant rs34080917 [ dbSNP | Ensembl ].
VAR_048831

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei217 – 27660CFCFE…VPGYN → ASKLHRVYVLESWHL in isoform 2. 1 PublicationVSP_046360Add
BLAST

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS11583.1. [Q9Y6N1-1]
CCDS58579.1. [Q9Y6N1-2]
RefSeqiNP_004366.1. NM_004375.3. [Q9Y6N1-1]
XP_006721767.1. XM_006721704.1. [Q9Y6N1-1]
XP_006721768.1. XM_006721705.1. [Q9Y6N1-1]
UniGeneiHs.591171.

Genome annotation databases

EnsembliENST00000299335; ENSP00000299335; ENSG00000166260. [Q9Y6N1-1]
ENST00000572558; ENSP00000459302; ENSG00000166260. [Q9Y6N1-2]
ENST00000576370; ENSP00000459901; ENSG00000166260. [Q9Y6N1-1]
GeneIDi1353.
KEGGihsa:1353.
UCSCiuc010wng.1. human. [Q9Y6N1-1]

Polymorphism databases

DMDMi60416378.

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS11583.1. [Q9Y6N1-1]
CCDS58579.1. [Q9Y6N1-2]
RefSeqiNP_004366.1. NM_004375.3. [Q9Y6N1-1]
XP_006721767.1. XM_006721704.1. [Q9Y6N1-1]
XP_006721768.1. XM_006721705.1. [Q9Y6N1-1]
UniGeneiHs.591171.

3D structure databases

ProteinModelPortaliQ9Y6N1.
SMRiQ9Y6N1. Positions 145-262.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107746. 3 interactions.
IntActiQ9Y6N1. 3 interactions.
STRINGi9606.ENSP00000299335.

PTM databases

PhosphoSiteiQ9Y6N1.

Polymorphism databases

DMDMi60416378.

Proteomic databases

MaxQBiQ9Y6N1.
PaxDbiQ9Y6N1.
PRIDEiQ9Y6N1.

Protocols and materials databases

DNASUi1353.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000299335; ENSP00000299335; ENSG00000166260. [Q9Y6N1-1]
ENST00000572558; ENSP00000459302; ENSG00000166260. [Q9Y6N1-2]
ENST00000576370; ENSP00000459901; ENSG00000166260. [Q9Y6N1-1]
GeneIDi1353.
KEGGihsa:1353.
UCSCiuc010wng.1. human. [Q9Y6N1-1]

Organism-specific databases

CTDi1353.
GeneCardsiGC17M053029.
HGNCiHGNC:2261. COX11.
HPAiHPA044020.
MIMi603648. gene.
neXtProtiNX_Q9Y6N1.
PharmGKBiPA26777.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiCOG3175.
GeneTreeiENSGT00390000007512.
HOGENOMiHOG000264965.
HOVERGENiHBG051085.
InParanoidiQ9Y6N1.
KOiK02258.
OMAiRMARVDT.
OrthoDBiEOG7JMGFB.
PhylomeDBiQ9Y6N1.
TreeFamiTF105072.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.
REACT_267716. Orphan transporters.

Miscellaneous databases

GenomeRNAii1353.
NextBioi5483.
PROiQ9Y6N1.
SOURCEiSearch...

Gene expression databases

BgeeiQ9Y6N1.
CleanExiHS_COX11.
ExpressionAtlasiQ9Y6N1. baseline and differential.
GenevestigatoriQ9Y6N1.

Family and domain databases

Gene3Di2.60.370.10. 1 hit.
HAMAPiMF_00155. CtaG.
InterProiIPR023471. CtaG/Cox11_dom.
IPR007533. Cyt_c_oxidase_assmbl_CtaG.
[Graphical view]
PANTHERiPTHR21320. PTHR21320. 1 hit.
PfamiPF04442. CtaG_Cox11. 1 hit.
[Graphical view]
SUPFAMiSSF110111. SSF110111. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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. 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.

Entry informationi

Entry nameiCOX11_HUMAN
AccessioniPrimary (citable) accession number: Q9Y6N1
Secondary accession number(s): D3DTY5
, I3L220, Q6FHB7, Q9BRX0, Q9UME8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 30, 2000
Last sequence update: March 1, 2005
Last modified: January 7, 2015
This is version 122 of the entry and version 3 of the sequence. [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

Documents

  1. Human chromosome 17
    Human chromosome 17: 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

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.