WISP3, Polyclonal Antibody

WNT1-inducible-signaling pathway protein 3; WISP-3; CCN family member 6; WISP3; CCN6

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit Ig

Rabbit

This WISP3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 305-335 amino acids from the Central region of human WISP3.

Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Vial Concentration: 2 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-WISP3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

WISP3 is 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. WISP3 is overexpressed in colon tumors. It may be downstream in the WNT1 signaling pathway that is relevant to malignant transformation. Mutations of the WISP3 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.

Immunology; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC)

WB~~1:1000

Western blot analysis of anti-WISP3 Antibody (Center) in HepG2 cell line lysates (35ug/lane). WISP3 (arrow) was detected using the purified Pab.

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.

39293

WNT1 inducible signaling pathway protein 3

WNT1-inducible-signaling pathway protein 3

WNT1-inducible-signaling pathway protein 3

CCN6; WISP-3  [Similar Products]

WISP3_HUMAN

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]

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

Clark, H.F., et al., Genome Res. 13(10):2265-2270 (2003).
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).

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