It is a member of the CCN family (CCN intercellular signaling protein) of secreted, extracellular matrix (ECM)-associated signaling matricellular proteins. The CCN acronym is derived from the first three members of the family identified, namely CYR61 (cysteine-rich angiogenic inducer 61, or CCN1), CTGF (connective tissue growth factor, or CCN2), and NOV (nephroblastoma overexpressed, or CCN3). These proteins, together with WISP1 (CCN4), and WISP2 (CCN5) comprise the six-member CCN family in vertebrates. CCN proteins characteristically contain an N-terminal secretory signal peptide followed by four structurally distinct domains with homologies to insulin-like growth factor binding protein (IGFBP), von Willebrand type C repeats (vWC), thrombospondin type 1 repeat (TSR), and a cysteine knot motif within the C-terminal (CT) domain.
Functionedit
The CCN family of proteins regulates diverse cellular functions, including cell adhesion, migration, proliferation, survival, and differentiation.[7][8][9]
Clinical significanceedit
Mutations in the human WISP3 gene are associated with progressive pseudorheumatoid dysplasia, a juvenile onset autosomal recessive skeletal disorder, indicating that the gene is essential for normal postnatal skeletal growth and cartilage homeostasis.[10] However, mice with WISP3 knockout or overexpression are normal and suffer no apparent developmental defect.[11][12] Loss of WISP3 expression is associated with aggressive inflammatory breast cancer and breast cancer with axillary lymph node metastasis, suggesting that WISP3/CCN6 may function as a suppressor of breast cancer growth and metastasis.[6]
Referencesedit
^ abcGRCh38: Ensembl release 89: ENSG00000112761 – Ensembl, May 2017
^ abcGRCm38: Ensembl release 89: ENSMUSG00000062074 – Ensembl, May 2017
^"Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^"Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^Jun JI, Lau LF (December 2011). "Taking aim at the extracellular matrix: CCN proteins as emerging therapeutic targets". Nat Rev Drug Discov. 10 (12): 945–63. doi:10.1038/nrd3599. PMC3663145. PMID 22129992.
^ abHuang W, Pal A, Kleer CG (March 2012). "On how CCN6 suppresses breast cancer growth and invasion". J Cell Commun Signal. 6 (1): 5–10. doi:10.1007/s12079-011-0148-9. PMC3271195. PMID 21842227.
^Chen CC, Lau LF (April 2009). "Functions and mechanisms of action of CCN matricellular proteins". Int. J. Biochem. Cell Biol. 41 (4): 771–83. doi:10.1016/j.biocel.2008.07.025. PMC2668982. PMID 18775791.
^Holbourn KP, Acharya KR, Perbal B (October 2008). "The CCN family of proteins: structure-function relationships". Trends Biochem. Sci. 33 (10): 461–73. doi:10.1016/j.tibs.2008.07.006. PMC2683937. PMID 18789696.
^Leask A, Abraham DJ (December 2006). "All in the CCN family: essential matricellular signaling modulators emerge from the bunker". J. Cell Sci. 119 (Pt 23): 4803–10. doi:10.1242/jcs.03270. PMID 17130294.
^Hurvitz JR, Suwairi WM, Van Hul W, El-Shanti H, Superti-Furga A, Roudier J, Holderbaum D, Pauli RM, Herd JK, Van Hul EV, Rezai-Delui H, Legius E, Le Merrer M, Al-Alami J, Bahabri SA, Warman ML (September 1999). "Mutations in the CCN gene family member WISP3 cause progressive pseudorheumatoid dysplasia". Nat. Genet. 23 (1): 94–8. doi:10.1038/12699. PMID 10471507. S2CID 31389994.
^Kutz WE, Gong Y, Warman ML (January 2005). "WISP3, the gene responsible for the human skeletal disease progressive pseudorheumatoid dysplasia, is not essential for skeletal function in mice". Mol. Cell. Biol. 25 (1): 414–21. doi:10.1128/MCB.25.1.414-421.2005. PMC538768. PMID 15601861.
^Nakamura Y, Cui Y, Fernando C, Kutz WE, Warman ML (June 2009). "Normal growth and development in mice over-expressing the CCN family member WISP3". J Cell Commun Signal. 3 (2): 105–13. doi:10.1007/s12079-009-0040-z. PMC2721080. PMID 19401829.
This article on a gene on human chromosome 6 is a stub. You can help Wikipedia by expanding it.
wnt1, inducible, signaling, pathway, protein, wisp3, also, named, ccn6, matricellular, protein, that, humans, encoded, wisp3, gene, ccn6identifiersaliasesccn6, libc, ppac, wisp, wnt1, inducible, signaling, pathway, protein, wisp3, cellular, communication, netw. WNT1 inducible signaling pathway protein 3 5 6 WISP3 also named CCN6 is a matricellular protein that in humans is encoded by the WISP3 gene CCN6IdentifiersAliasesCCN6 LIBC PPAC PPD WISP 3 WNT1 inducible signaling pathway protein 3 WISP3 cellular communication network factor 6 PPRDExternal IDsOMIM 603400 MGI 2685581 HomoloGene 77038 GeneCards CCN6Gene location Human Chr Chromosome 6 human 1 Band6q21Start112 054 075 bp 1 End112 069 686 bp 1 Gene location Mouse Chr Chromosome 10 mouse 2 Band10 10 B1Start39 150 970 bp 2 End39 163 794 bp 2 RNA expression patternBgeeHumanMouse ortholog Top expressed intibiaspermcorpus epididymiscerebellar hemisphereright uterine tubeglomerulusmetanephric glomerulusskin of abdomenputamenkidneyTop expressed insecondary oocytethymusbone marrowlensganglionic eminenceadrenal glandwhite adipose tissuespermatocyteblastocysthypothalamusMore reference expression dataBioGPSMore reference expression dataGene ontologyMolecular functioninsulin like growth factor binding growth factor activity integrin binding heparin bindingCellular componentextracellular region extracellular space endoplasmic reticulum extracellular matrix mitochondrionBiological processcell cell signaling regulation of cell growth signal transduction cell adhesion negative regulation of cell death regulation of signaling receptor activity regulation of mitochondrial membrane potential regulation of reactive oxygen species biosynthetic processSources Amigo QuickGOOrthologsSpeciesHumanMouseEntrez8838327743EnsemblENSG00000112761ENSMUSG00000062074UniProtO95389D3Z5L9RefSeq mRNA NM 003880NM 130396NM 198239NM 001127376RefSeq protein NP 003871NP 937882NP 001120848Location UCSC Chr 6 112 05 112 07 MbChr 10 39 15 39 16 MbPubMed search 3 4 WikidataView Edit HumanView Edit Mouse Contents 1 Structure 2 Function 3 Clinical significance 4 ReferencesStructure editIt is a member of the CCN family CCN intercellular signaling protein of secreted extracellular matrix ECM associated signaling matricellular proteins The CCN acronym is derived from the first three members of the family identified namely CYR61 cysteine rich angiogenic inducer 61 or CCN1 CTGF connective tissue growth factor or CCN2 and NOV nephroblastoma overexpressed or CCN3 These proteins together with WISP1 CCN4 and WISP2 CCN5 comprise the six member CCN family in vertebrates CCN proteins characteristically contain an N terminal secretory signal peptide followed by four structurally distinct domains with homologies to insulin like growth factor binding protein IGFBP von Willebrand type C repeats vWC thrombospondin type 1 repeat TSR and a cysteine knot motif within the C terminal CT domain Function editThe CCN family of proteins regulates diverse cellular functions including cell adhesion migration proliferation survival and differentiation 7 8 9 Clinical significance editMutations in the human WISP3 gene are associated with progressive pseudorheumatoid dysplasia a juvenile onset autosomal recessive skeletal disorder indicating that the gene is essential for normal postnatal skeletal growth and cartilage homeostasis 10 However mice with WISP3 knockout or overexpression are normal and suffer no apparent developmental defect 11 12 Loss of WISP3 expression is associated with aggressive inflammatory breast cancer and breast cancer with axillary lymph node metastasis suggesting that WISP3 CCN6 may function as a suppressor of breast cancer growth and metastasis 6 References edit a b c GRCh38 Ensembl release 89 ENSG00000112761 Ensembl May 2017 a b c GRCm38 Ensembl release 89 ENSMUSG00000062074 Ensembl May 2017 Human PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Mouse PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Jun JI Lau LF December 2011 Taking aim at the extracellular matrix CCN proteins as emerging therapeutic targets Nat Rev Drug Discov 10 12 945 63 doi 10 1038 nrd3599 PMC 3663145 PMID 22129992 a b Huang W Pal A Kleer CG March 2012 On how CCN6 suppresses breast cancer growth and invasion J Cell Commun Signal 6 1 5 10 doi 10 1007 s12079 011 0148 9 PMC 3271195 PMID 21842227 Chen CC Lau LF April 2009 Functions and mechanisms of action of CCN matricellular proteins Int J Biochem Cell Biol 41 4 771 83 doi 10 1016 j biocel 2008 07 025 PMC 2668982 PMID 18775791 Holbourn KP Acharya KR Perbal B October 2008 The CCN family of proteins structure function relationships Trends Biochem Sci 33 10 461 73 doi 10 1016 j tibs 2008 07 006 PMC 2683937 PMID 18789696 Leask A Abraham DJ December 2006 All in the CCN family essential matricellular signaling modulators emerge from the bunker J Cell Sci 119 Pt 23 4803 10 doi 10 1242 jcs 03270 PMID 17130294 Hurvitz JR Suwairi WM Van Hul W El Shanti H Superti Furga A Roudier J Holderbaum D Pauli RM Herd JK Van Hul EV Rezai Delui H Legius E Le Merrer M Al Alami J Bahabri SA Warman ML September 1999 Mutations in the CCN gene family member WISP3 cause progressive pseudorheumatoid dysplasia Nat Genet 23 1 94 8 doi 10 1038 12699 PMID 10471507 S2CID 31389994 Kutz WE Gong Y Warman ML January 2005 WISP3 the gene responsible for the human skeletal disease progressive pseudorheumatoid dysplasia is not essential for skeletal function in mice Mol Cell Biol 25 1 414 21 doi 10 1128 MCB 25 1 414 421 2005 PMC 538768 PMID 15601861 Nakamura Y Cui Y Fernando C Kutz WE Warman ML June 2009 Normal growth and development in mice over expressing the CCN family member WISP3 J Cell Commun Signal 3 2 105 13 doi 10 1007 s12079 009 0040 z PMC 2721080 PMID 19401829 nbsp This article on a gene on human chromosome 6 is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title WNT1 inducible signaling pathway protein 3 amp oldid 1142709314, wikipedia, wiki, book, books, library,
