Rabbit anti-Human WISP3 Polyclonal Antibody | anti-WISP3 antibody
WISP3 Antibody (Center)
Western Blot (WB)
(Western blot analysis of anti-WISP3 Antibody (Center) in HepG2 cell line lysates (35ug/lane). WISP3 (arrow) was detected using the purified Pab.)
Immunohistochemistry (IHC)
(Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma.)
Tanaka, S., et al., Gastroenterology 123(1):392-393 (2002).
Kleer, C.G., et al., Oncogene 21(20):3172-3180 (2002).
Hurvitz, J.R., et al., Nat. Genet. 23(1):94-98 (1999).
Pennica, D., et al., Proc. Natl. Acad. Sci. U.S.A. 95(25):14717-14722 (1998).
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the WNT1 inducible signaling pathway (WISP) protein subfamily, which belongs to the connective tissue growth factor (CTGF) family. WNT1 is a member of a family of cysteine-rich, glycosylated signaling proteins that mediate diverse developmental processes. The CTGF family members are characterized by four conserved cysteine-rich domains: insulin-like growth factor-binding domain, von Willebrand factor type C module, thrombospondin domain and C-terminal cystine knot-like domain. This gene is overexpressed in colon tumors. It may be downstream in the WNT1 signaling pathway that is relevant to malignant transformation. Mutations of this gene are associated with progressive pseudorheumatoid dysplasia, an autosomal recessive skeletal disorder, indicating that the gene is essential for normal postnatal skeletal growth and cartilage homeostasis. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]
Uniprot Description
WISP3: Appears to be required for normal postnatal skeletal growth and cartilage homeostasis. Defects in WISP3 are the cause of progressive pseudorheumatoid arthropathy of childhood (PPAC). 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. Belongs to the CCN family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted, signal peptide; Cell cycle regulation; Secreted
Chromosomal Location of Human Ortholog: 6q21
Cellular Component: extracellular space; proteinaceous extracellular matrix
Molecular Function: heparin binding; integrin binding; insulin-like growth factor binding; growth factor activity
Biological Process: cell-cell signaling; regulation of cell growth; signal transduction; cell adhesion
Disease: Arthropathy, Progressive Pseudorheumatoid, Of Childhood