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Q14626 (I11RA_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 107. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Web links·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Interleukin-11 receptor subunit alpha

Short name=IL-11 receptor subunit alpha
Short name=IL-11R subunit alpha
Short name=IL-11R-alpha
Short name=IL-11RA
Gene names
Name:IL11RA
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length422 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Receptor for interleukin-11. The receptor systems for IL6, LIF, OSM, CNTF, IL11 and CT1 can utilize IL6ST for initiating signal transmission. The IL11/IL11RA/IL6ST complex may be involved in the control of proliferation and/or differentiation of skeletogenic progenitor or other mesenchymal cells. Essential for the normal development of craniofacial bones and teeth. Restricts suture fusion and tooth number. Ref.9

Subunit structure

On ligand binding, forms a multimer complex with IL6ST/gp130.

Subcellular location

Membrane; Single-pass type I membrane protein.

Tissue specificity

Expressed in a number of cell lines, including the myelogenous leukemia cell line K-562, the megakaryocytic leukemia cell line M-07e, the erythroleukemia cell line TF-1, and the osteosarcoma cell lines, MG-63 and SaOS-2. Also expressed in normal and malignant prostate epithelial cell lines. Expression levels are increased in prostate carcinoma. Ref.8

Involvement in disease

Craniosynostosis and dental anomalies (CRSDA) [MIM:614188]: A disorder characterized by craniosynostosis, maxillary hypoplasia, and dental anomalies, including malocclusion, delayed and ectopic tooth eruption, and/or supernumerary teeth. Some patients also display minor digit anomalies, such as syndactyly and/or clinodactyly.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.9

Sequence similarities

Belongs to the type I cytokine receptor family. Type 3 subfamily.

Contains 2 fibronectin type-III domains.

Contains 1 Ig-like C2-type (immunoglobulin-like) domain.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform HCR1 (identifier: Q14626-1)

Also known as: Membrane form;

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 HCR2 (identifier: Q14626-2)

Also known as: Soluble form; sIL11RA;

The sequence of this isoform differs from the canonical sequence as follows:
     391-422: Missing.
Note: Lacks the entire cytoplasmic domain.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Signal peptide1 – 2222 Potential
Chain23 – 422400Interleukin-11 receptor subunit alpha
PRO_0000010913

Regions

Topological domain24 – 370347Extracellular Potential
Transmembrane371 – 39121Helical; Potential
Topological domain392 – 42231Cytoplasmic Potential
Domain27 – 11084Ig-like C2-type
Domain112 – 219108Fibronectin type-III 1
Domain220 – 31798Fibronectin type-III 2
Motif304 – 3085WSXWS motif

Amino acid modifications

Glycosylation1271N-linked (GlcNAc...) Potential
Glycosylation1941N-linked (GlcNAc...) Potential
Disulfide bond48 ↔ 94 By similarity
Disulfide bond120 ↔ 130 By similarity
Disulfide bond170 ↔ 180 By similarity

Natural variations

Alternative sequence391 – 42232Missing in isoform HCR2.
VSP_011879
Natural variant651P → T. Ref.4
Corresponds to variant rs11575589 [ dbSNP | Ensembl ].
VAR_019821
Natural variant2211P → R in CRSDA. Ref.9
VAR_066666
Natural variant2451S → C in CRSDA. Ref.9
VAR_066667
Natural variant2961R → W in CRSDA; renders the receptor unable to mediate the IL11 signal. Ref.9
VAR_066668
Natural variant3081S → STWS in CRSDA.
VAR_066669
Natural variant3951R → W. Ref.4
Corresponds to variant rs11575580 [ dbSNP | Ensembl ].
VAR_019822

Sequences

Sequence LengthMass (Da)Tools
Isoform HCR1 (Membrane form) [UniParc].

Last modified November 23, 2004. Version 2.
Checksum: 1F8BC05C139FC326

FASTA42245,222
        10         20         30         40         50         60 
MSSSCSGLSR VLVAVATALV SASSPCPQAW GPPGVQYGQP GRSVKLCCPG VTAGDPVSWF 

        70         80         90        100        110        120 
RDGEPKLLQG PDSGLGHELV LAQADSTDEG TYICQTLDGA LGGTVTLQLG YPPARPVVSC 

       130        140        150        160        170        180 
QAADYENFSC TWSPSQISGL PTRYLTSYRK KTVLGADSQR RSPSTGPWPC PQDPLGAARC 

       190        200        210        220        230        240 
VVHGAEFWSQ YRINVTEVNP LGASTRLLDV SLQSILRPDP PQGLRVESVP GYPRRLRASW 

       250        260        270        280        290        300 
TYPASWPCQP HFLLKFRLQY RPAQHPAWST VEPAGLEEVI TDAVAGLPHA VRVSARDFLD 

       310        320        330        340        350        360 
AGTWSTWSPE AWGTPSTGTI PKEIPAWGQL HTQPEVEPQV DSPAPPRPSL QPHPRLLDHR 

       370        380        390        400        410        420 
DSVEQVAVLA SLGILSFLGL VAGALALGLW LRLRRGGKDG SPKPGFLASV IPVDRRPGAP 


NL 

« Hide

Isoform HCR2 (Soluble form) (sIL11RA) [UniParc].

Checksum: 847F8C6F5F52248D
Show »

FASTA39041,842

References

« Hide 'large scale' references
[1]"Molecular cloning of two isoforms of a receptor for the human hematopoietic cytokine interleukin-11."
Cherel M., Sorel M., Lebeau B., Dubois S., Moreau J.-F., Bataille R., Minvielle S., Jacques Y.
Blood 86:2534-2540(1995) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORMS HCR1 AND HCR2).
Tissue: Muscle.
[2]"Molecular cloning and characterization of the human interleukin-11 receptor alpha-chain gene, IL11RA, located on chromosome 9p13."
Van Leuven F., Stas L., Hilliker C., Miyake Y., Bilinski P., Gossler A.
Genomics 31:65-70(1996) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] (ISOFORM HCR1).
[3]"Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., LaBaer J., Lin Y., Phelan M., Farmer A.
Submitted (AUG-2003) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM HCR1).
[4]SeattleSNPs variation discovery resource
Submitted (JAN-2004) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANTS THR-65 AND TRP-395.
[5]"DNA sequence and analysis of human chromosome 9."
Humphray S.J., Oliver K., Hunt A.R., Plumb R.W., Loveland J.E., Howe K.L., Andrews T.D., Searle S., Hunt S.E., Scott C.E., Jones M.C., Ainscough R., Almeida J.P., Ambrose K.D., Ashwell R.I.S., Babbage A.K., Babbage S., Bagguley C.L. expand/collapse author list , Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beasley H., Beasley O., Bird C.P., Bray-Allen S., Brown A.J., Brown J.Y., Burford D., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Chen Y., Clarke G., Clark S.Y., Clee C.M., Clegg S., Collier R.E., Corby N., Crosier M., Cummings A.T., Davies J., Dhami P., Dunn M., Dutta I., Dyer L.W., Earthrowl M.E., Faulkner L., Fleming C.J., Frankish A., Frankland J.A., French L., Fricker D.G., Garner P., Garnett J., Ghori J., Gilbert J.G.R., Glison C., Grafham D.V., Gribble S., Griffiths C., Griffiths-Jones S., Grocock R., Guy J., Hall R.E., Hammond S., Harley J.L., Harrison E.S.I., Hart E.A., Heath P.D., Henderson C.D., Hopkins B.L., Howard P.J., Howden P.J., Huckle E., Johnson C., Johnson D., Joy A.A., Kay M., Keenan S., Kershaw J.K., Kimberley A.M., King A., Knights A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C., Lloyd D.M., Lovell J., Martin S., Mashreghi-Mohammadi M., Matthews L., McLaren S., McLay K.E., McMurray A., Milne S., Nickerson T., Nisbett J., Nordsiek G., Pearce A.V., Peck A.I., Porter K.M., Pandian R., Pelan S., Phillimore B., Povey S., Ramsey Y., Rand V., Scharfe M., Sehra H.K., Shownkeen R., Sims S.K., Skuce C.D., Smith M., Steward C.A., Swarbreck D., Sycamore N., Tester J., Thorpe A., Tracey A., Tromans A., Thomas D.W., Wall M., Wallis J.M., West A.P., Whitehead S.L., Willey D.L., Williams S.A., Wilming L., Wray P.W., Young L., Ashurst J.L., Coulson A., Blocker H., Durbin R.M., Sulston J.E., Hubbard T., Jackson M.J., Bentley D.R., Beck S., Rogers J., Dunham I.
Nature 429:369-374(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]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. expand/collapse author list , 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].
[7]"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] (ISOFORM HCR1).
Tissue: Brain.
[8]"Increased expression of the interleukin-11 receptor and evidence of STAT3 activation in prostate carcinoma."
Campbell C.L., Jiang Z., Savarese D.M., Savarese T.M.
Am. J. Pathol. 158:25-32(2001) [PubMed] [Europe PMC] [Abstract]
Cited for: TISSUE SPECIFICITY.
[9]"Inactivation of IL11 signaling causes craniosynostosis, delayed tooth eruption, and supernumerary teeth."
Nieminen P., Morgan N.V., Fenwick A.L., Parmanen S., Veistinen L., Mikkola M.L., van der Spek P.J., Giraud A., Judd L., Arte S., Brueton L.A., Wall S.A., Mathijssen I.M., Maher E.R., Wilkie A.O., Kreiborg S., Thesleff I.
Am. J. Hum. Genet. 89:67-81(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, VARIANTS CRSDA ARG-221; CYS-245; TRP-296 AND THR-TRP-SER-308 INS, CHARACTERIZATION OF VARIANT CRSDA TRP-296.
+Additional computationally mapped references.

Web resources

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
Z38102 mRNA. Translation: CAA86224.1.
Z46595 mRNA. Translation: CAA86570.1.
U32323 Genomic DNA. Translation: AAB36491.1.
U32324 mRNA. Translation: AAB36492.1.
BT009864 mRNA. Translation: AAP88866.1.
AY532110 Genomic DNA. Translation: AAS00093.1.
AL162231 Genomic DNA. Translation: CAH69857.1.
CH471071 Genomic DNA. Translation: EAW58423.1.
BC003110 mRNA. Translation: AAH03110.1.
CCDSCCDS6567.1. [Q14626-1]
PIRI37891.
RefSeqNP_001136256.1. NM_001142784.2. [Q14626-1]
UniGeneHs.591088.

3D structure databases

ProteinModelPortalQ14626.
SMRQ14626. Positions 22-317.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

DIPDIP-3776N.
STRING9606.ENSP00000326500.

Chemistry

ChEMBLCHEMBL2050.
DrugBankDB00038. Oprelvekin.

PTM databases

PhosphoSiteQ14626.

Polymorphism databases

DMDM55976300.

Proteomic databases

PaxDbQ14626.
PRIDEQ14626.

Protocols and materials databases

DNASU3590.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000318041; ENSP00000326500; ENSG00000137070. [Q14626-1]
ENST00000378817; ENSP00000368094; ENSG00000137070. [Q14626-2]
ENST00000441545; ENSP00000394391; ENSG00000137070. [Q14626-1]
ENST00000555003; ENSP00000450565; ENSG00000137070. [Q14626-1]
ENST00000602473; ENSP00000473647; ENSG00000137070. [Q14626-2]
GeneID3590.
KEGGhsa:3590.
UCSCuc003zvi.4. human. [Q14626-1]

Organism-specific databases

CTD3590.
GeneCardsGC09P034650.
HGNCHGNC:5967. IL11RA.
HPACAB032830.
HPA013162.
HPA036652.
MIM600939. gene.
614188. phenotype.
neXtProtNX_Q14626.
Orphanet284149. Craniosynostosis and dental anomalies.
PharmGKBPA29782.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG28915.
HOGENOMHOG000231670.
HOVERGENHBG063427.
InParanoidQ14626.
KOK05056.
OMASQYRINV.
OrthoDBEOG72NRQF.
PhylomeDBQ14626.
TreeFamTF331210.

Enzyme and pathway databases

SignaLinkQ14626.

Gene expression databases

ArrayExpressQ14626.
BgeeQ14626.
CleanExHS_IL11RA.
GenevestigatorQ14626.

Family and domain databases

Gene3D2.60.40.10. 3 hits.
InterProIPR003961. Fibronectin_type3.
IPR003530. Hematopoietin_rcpt_L_F3_CS.
IPR007110. Ig-like_dom.
IPR013783. Ig-like_fold.
IPR003599. Ig_sub.
[Graphical view]
SMARTSM00060. FN3. 2 hits.
SM00409. IG. 1 hit.
[Graphical view]
SUPFAMSSF49265. SSF49265. 2 hits.
PROSITEPS50853. FN3. 2 hits.
PS01354. HEMATOPO_REC_L_F3. 1 hit.
PS50835. IG_LIKE. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSIL11RA. human.
GeneWikiInterleukin_11_receptor_alpha_subunit.
GenomeRNAi3590.
NextBio14027.
PROQ14626.
SOURCESearch...

Entry information

Entry nameI11RA_HUMAN
AccessionPrimary (citable) accession number: Q14626
Secondary accession number(s): Q16542, Q5VZ80, Q7KYJ7
Entry history
Integrated into UniProtKB/Swiss-Prot: November 23, 2004
Last sequence update: November 23, 2004
Last modified: July 9, 2014
This is version 107 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant 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 polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 9

Human chromosome 9: entries, gene names and cross-references to MIM