background:
Wnt-induced secreted protein (WISP)-1, WISP-2 and WISP-3 are members of the CCN family of growth factors, which include connective tissue growth factor (CTGF) and Cyr61. WISP-1, WISP-2 and WISP-3 share significant sequence similarity, including four conserved cysteine-rich domains, and they are believed to function as dimers in their active forms. WISP-1 expression is observed in various tissues including adult heart, kidney and spleen, while WISP-2 expression predominates in skeletal muscle, colon and ovary. Both WISP-1 and WISP-2 are upregulated in cells transformed with the proto-oncogene Wnt-1, and they are also more highly expressed in human colon tumors, suggesting that these proteins may participate in tumor development. WISP-3 is involved in normal post-natal skeletal growth, and it is also implicated in the development of the autosomal recessive skeletal disorder progressive pseudorheumatoid dysplasia, which affects cartilage homeostasis by disrupting the growth of chondrocyte and normal cell columnar organization.
Function:
Appears to be required for normal postnatal skeletal growth and cartilage homeostasis.
Subcellular Location:
Secreted.
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.
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.
Similarity:
Belongs to the CCN family.
Contains 1 CTCK (SLCterminal cystine knot-like) domain.
Contains 1 IGFBP N-terminal domain.
Contains 1 TSP type-1 domain.
Database links:
Entrez Gene: 8838 Human
Entrez Gene: 327743 Mouse
Entrez Gene: 499461 Rat
Omim: 603400 Human
SwissProt: O95389 Human
SwissProt: D3Z5L9 Mouse
Unigene: 558428 Human
Unigene: 208111 Mouse
Unigene: 141375 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
|
|
