WNT1-inducible-signaling pathway protein 3

CCN6
Identifiers
Aliases	CCN6, LIBC, PPAC, PPD, WISP-3, WNT1 inducible signaling pathway protein 3, WISP3, cellular communication network factor 6, PPRD
External IDs	OMIM: 603400; MGI: 2685581; HomoloGene: 77038; GeneCards: CCN6; OMA:CCN6 - orthologs
Gene location (Human) Chr. Chromosome 6 (human)[1] Band 6q21 Start 112,054,075 bp[1] End 112,069,686 bp[1]
Gene location (Mouse) Chr. Chromosome 10 (mouse)[2] Band 10|10 B1 Start 39,150,970 bp[2] End 39,163,794 bp[2]
RNA expression pattern Bgee Human Mouse (ortholog) Top expressed in tibia testicle cartilage tissue sperm corpus epididymis cerebellar hemisphere right hemisphere of cerebellum right uterine tube right testis skin of leg Top expressed in secondary oocyte primary oocyte zygote neural layer of retina thymus embryo embryo bone marrow granulocyte lens More reference expression data BioGPS More reference expression data
Gene ontology Molecular function insulin-like growth factor binding growth factor activity integrin binding heparin binding Cellular component extracellular region extracellular space endoplasmic reticulum extracellular matrix mitochondrion Biological process cell-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 process Sources:Amigo / QuickGO
Orthologs Species Human Mouse Entrez 8838 327743 Ensembl ENSG00000112761 ENSMUSG00000062074 UniProt O95389 D3Z5L9 RefSeq (mRNA) NM_003880 NM_130396 NM_198239 NM_001127376 RefSeq (protein) NP_003871 NP_937882 NP_001120848 Location (UCSC) Chr 6: 112.05 – 112.07 Mb Chr 10: 39.15 – 39.16 Mb PubMed search [3] [4]
Wikidata
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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.

Structure

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.

Function

The CCN family of proteins regulates diverse cellular functions, including cell adhesion, migration, proliferation, survival, and differentiation.^[7][8][9]

Clinical significance

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]

References