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Reviewed, UniProtKB/Swiss-Prot O95389 (WISP3_HUMAN)

Last modified June 16, 2009. Version 73. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data | Customize display text xml rdf/xml gff fasta
Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Web resources · Cross-references · Entry information · Relevant documents

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Names and origin

Protein namesRecommended name:
    WNT1-inducible-signaling pathway protein 3
      Short name=WISP-3
Gene names
Name: WISP3
Synonyms: CCN6
ORF Names: UNQ462/PRO790/PRO956
OrganismHomo sapiens (Human)
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo

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Protein attributes

Sequence length354 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level.

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General annotation (Comments)

Function

Appears to be required for normal postnatal skeletal growth and cartilage homeostasis.

Subcellular location

Secreted Probable.

Tissue specificity

Predominant expression in adult kidney and testis and fetal kidney. Weaker expression found in placenta, ovary, prostate and small intestine. Also expressed in skeletally-derived cells such as synoviocytes and articular cartilage chondrocytes.

Involvement in disease

Defects in WISP3 are the cause of progressive pseudorheumatoid arthropathy of childhood (PPAC) [MIM:208230]. PPAC is an autosomal recessive disorder characterized by stiffness and swelling of joints, motor weakness and joint contractures. Signs and symptoms of the disease develop typically between three and eight years of age. This progressive disease is a primary disorder of articular cartilage with continued cartilage loss and destructive bone changes with aging. Ref.4

Sequence similarities

Belongs to the CCN family.

Contains 1 CTCK (C-terminal cystine knot-like) domain.

Contains 1 IGFBP N-terminal domain.

Contains 1 TSP type-1 domain.

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Ontologies

Keywords
   Cellular componentSecreted
   Coding sequence diversityPolymorphism
   DiseaseDisease mutation
   DomainSignal
   Molecular functionGrowth factor
   PTMDisulfide bond
Glycoprotein
Gene Ontology (GO)
   Biological processcell-cell signaling Ref.1

Traceable author statement. Source: ProtInc

regulation of cell growth

Inferred from electronic annotation. Source: InterPro

signal transduction Ref.1

Traceable author statement. Source: ProtInc

   Cellular componentextracellular region

Inferred from electronic annotation. Source: UniProtKB-SubCell

soluble fraction Ref.1

Traceable author statement. Source: ProtInc

   Molecular functiongrowth factor activity

Inferred from electronic annotation. Source: UniProtKB-KW

insulin-like growth factor binding

Inferred from electronic annotation. Source: InterPro

Complete GO annotation...

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Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Signal peptide1 – 2323 Potential
Chain24 – 354331WNT1-inducible-signaling pathway protein 3
PRO_0000014412

Regions

Domain44 – 11774IGFBP N-terminal
Domain208 – 25346TSP type-1
Domain268 – 34275CTCK

Amino acid modifications

Glycosylation1781N-linked (GlcNAc...) Potential
Glycosylation3081N-linked (GlcNAc...) Potential
Disulfide bond268 ↔ 305 By similarity
Disulfide bond285 ↔ 319 By similarity
Disulfide bond296 ↔ 335 By similarity
Disulfide bond299 ↔ 337 By similarity
Disulfide bond304 ↔ 341 By similarity

Natural variations

Natural variant561Q → H Common polymorphism. dbSNP rs1230345. Ref.4
VAR_016224
Natural variant601R → C: dbSNP rs17073260.
VAR_049567
Natural variant781C → R in PPAC. Ref.4
VAR_016225

Experimental info

Sequence conflict2001N → D in AAQ88715. Ref.2

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Sequences

Sequence LengthMass (Da)Tools
O95389-1 [UniParc].

Last modified May 1, 1999. Version 1.
Checksum: 67F48D0D5C2F5EE3

FASTA35439,293
        10         20         30         40         50         60 
MQGLLFSTLL LAGLAQFCCR VQGTGPLDTT PEGRPGEVSD APQRKQFCHW PCKCPQQKPR 

        70         80         90        100        110        120 
CPPGVSLVRD GCGCCKICAK QPGEICNEAD LCDPHKGLYC DYSVDRPRYE TGVCAYLVAV 

       130        140        150        160        170        180 
GCEFNQVHYH NGQVFQPNPL FSCLCVSGAI GCTPLFIPKL AGSHCSGAKG GKKSDQSNCS 

       190        200        210        220        230        240 
LEPLLQQLST SYKTMPAYRN LPLIWKKKCL VQATKWTPCS RTCGMGISNR VTNENSNCEM 

       250        260        270        280        290        300 
RKEKRLCYIQ PCDSNILKTI KIPKGKTCQP TFQLSKAEKF VFSGCSSTQS YKPTFCGICL 

       310        320        330        340        350 
DKRCCIPNKS KMITIQFDCP NEGSFKWKML WITSCVCQRN CREPGDIFSE LKIL

« Hide

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References

« Hide 'large scale' references
[1]"WISP genes are members of the connective tissue growth factor family that are up-regulated in wnt-1-transformed cells and aberrantly expressed in human colon tumors."
Pennica D., Swanson T.A., Welsh J.W., Roy M.A., Lawrence D.A., Lee J., Brush J., Taneyhill L.A., Deuel B., Lew M., Watanabe C., Cohen R.L., Melham M.F., Finley G.G., Quirke P., Goddard A.D., Hillan K.J., Gurney A.L., Botstein D., Levine A.J.
Proc. Natl. Acad. Sci. U.S.A. 95:14717-14722(1998) [PubMed: 9843955] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA].
Tissue: Bone marrow and Fetal kidney.
[2]"The secreted protein discovery initiative (SPDI), a large-scale effort to identify novel human secreted and transmembrane proteins: a bioinformatics assessment."
Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J., Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P., Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E. expand/collapse author list , Heldens S., Huang A., Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D., Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L., Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C., Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J., Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M.
Genome Res. 13:2265-2270(2003) [PubMed: 12975309] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
[3]"The DNA sequence and analysis of human chromosome 6."
Mungall A.J., Palmer S.A., Sims S.K., Edwards C.A., Ashurst J.L., Wilming L., Jones M.C., Horton R., Hunt S.E., Scott C.E., Gilbert J.G.R., Clamp M.E., Bethel G., Milne S., Ainscough R., Almeida J.P., Ambrose K.D., Andrews T.D. expand/collapse author list , Ashwell R.I.S., Babbage A.K., Bagguley C.L., Bailey J., Banerjee R., Barker D.J., Barlow K.F., Bates K., Beare D.M., Beasley H., Beasley O., Bird C.P., Blakey S.E., Bray-Allen S., Brook J., Brown A.J., Brown J.Y., Burford D.C., Burrill W., Burton J., Carder C., Carter N.P., Chapman J.C., Clark S.Y., Clark G., Clee C.M., Clegg S., Cobley V., Collier R.E., Collins J.E., Colman L.K., Corby N.R., Coville G.J., Culley K.M., Dhami P., Davies J., Dunn M., Earthrowl M.E., Ellington A.E., Evans K.A., Faulkner L., Francis M.D., Frankish A., Frankland J., French L., Garner P., Garnett J., Ghori M.J., Gilby L.M., Gillson C.J., Glithero R.J., Grafham D.V., Grant M., Gribble S., Griffiths C., Griffiths M.N.D., Hall R., Halls K.S., Hammond S., Harley J.L., Hart E.A., Heath P.D., Heathcott R., Holmes S.J., Howden P.J., Howe K.L., Howell G.R., Huckle E., Humphray S.J., Humphries M.D., Hunt A.R., Johnson C.M., Joy A.A., Kay M., Keenan S.J., Kimberley A.M., King A., Laird G.K., Langford C., Lawlor S., Leongamornlert D.A., Leversha M., Lloyd C.R., Lloyd D.M., Loveland J.E., Lovell J., Martin S., Mashreghi-Mohammadi M., Maslen G.L., Matthews L., McCann O.T., McLaren S.J., McLay K., McMurray A., Moore M.J.F., Mullikin J.C., Niblett D., Nickerson T., Novik K.L., Oliver K., Overton-Larty E.K., Parker A., Patel R., Pearce A.V., Peck A.I., Phillimore B.J.C.T., Phillips S., Plumb R.W., Porter K.M., Ramsey Y., Ranby S.A., Rice C.M., Ross M.T., Searle S.M., Sehra H.K., Sheridan E., Skuce C.D., Smith S., Smith M., Spraggon L., Squares S.L., Steward C.A., Sycamore N., Tamlyn-Hall G., Tester J., Theaker A.J., Thomas D.W., Thorpe A., Tracey A., Tromans A., Tubby B., Wall M., Wallis J.M., West A.P., White S.S., Whitehead S.L., Whittaker H., Wild A., Willey D.J., Wilmer T.E., Wood J.M., Wray P.W., Wyatt J.C., Young L., Younger R.M., Bentley D.R., Coulson A., Durbin R.M., Hubbard T., Sulston J.E., Dunham I., Rogers J., Beck S.
Nature 425:805-811(2003) [PubMed: 14574404] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[4]"Mutations in the CCN gene family member WISP3 cause progressive pseudorheumatoid dysplasia."
Hurvitz J.R., Suwairi W.M., Van Hul W., El-Shanti H., Superti-Furga A., Roudier J., Holderbaum D., Pauli R.M., Herd J.K., Van Hul E.V., Rezai-Delui H., Legius E., Le Merrer M., Al-Alami J., Bahabri S.A., Warman M.L.
Nat. Genet. 23:94-98(1999) [PubMed: 10471507] [Abstract]
Cited for: VARIANT PPAC ARG-78, VARIANT HIS-56.
+Additional computationally mapped references.

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Cross-references

Sequence databases

AF100781 mRNA. Translation: AAC96323.1.
AY358349 mRNA. Translation: AAQ88715.1. Different initiation.
AY358350 mRNA. Translation: AAQ88716.1.
AL512299, Z99289 Genomic DNA. Translation: CAI19998.1.
IPIIPI00022051.
RefSeqNP_003871.1.
NP_937882.1.
UniGeneHs.558428

3D structure databases

ModBaseSearch...

Proteomic databases

PRIDEO95389.

Genome annotation databases

EnsemblENSG00000112761. Homo sapiens. [Contig view]
GeneID8838.
KEGGhsa:8838.

Organism-specific databases

GeneCardsGC06P112481.
HGNCHGNC:12771. WISP3.
MIM208230. phenotype.
603400. gene.
Orphanet1159. Arthropathy progressive pseudorheumatoid of childhood.
252. Spondyloepimetaphyseal dysplasia.
PharmGKBPA37374.
GenAtlasSearch...

Phylogenomic databases

HOVERGENO95389.

Gene expression databases

BgeeO95389.
CleanExHS_WISP3.
GermOnlineENSG00000112761. Homo sapiens.

Family and domain databases

InterProIPR006208. Cys_knot.
IPR006207. Cys_knot_C.
IPR000867. IGFBP-like.
IPR012395. IGFBP_CNN.
IPR017891. Insulin_growth_fac-bd_prot_N.
IPR000884. Thrombospondin_1_rpt.
[Graphical view]
PfamPF00007. Cys_knot. 1 hit.
PF00219. IGFBP. 1 hit.
PF00090. TSP_1. 1 hit.
[Graphical view]
PIRSFPIRSF036495. IGFBP_rP_CNN. 1 hit.
SMARTSM00041. CT. 1 hit.
SM00121. IB. 1 hit.
SM00209. TSP1. 1 hit.
[Graphical view]
PROSITEPS01185. CTCK_1. False negative.
PS01225. CTCK_2. 1 hit.
PS00222. IGFBP_N_1. 1 hit.
PS51323. IGFBP_N_2. 1 hit.
PS50092. TSP1. 1 hit.
[Graphical view]
ProtoNetSearch...

Other Resources

NextBio33172.
SOURCESearch...

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Entry information

Entry nameWISP3_HUMAN
AccessionPrimary (citable) accession number: O95389
Secondary accession number(s): Q6UXH6
Entry history
Integrated into UniProtKB/Swiss-Prot: August 15, 2003
Last sequence update: May 1, 1999
Last modified: June 16, 2009
This is version 73 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation projectHPI (Human Proteome Initiative)

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Relevant documents

Human chromosome 6

Human chromosome 6: entries, gene names and cross-references to MIM

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

SIMILARITY comments

Index of protein domains and families

Names and origin · Protein attributes · General annotation (Comments) · Ontologies · Sequence annotation (Features) · Sequences · References · Web resources · Cross-references · Entry information · Relevant documents