WISP3 / PPD, Polyclonal Antibody

Anti-WISP3 / PPD Antibody (aa320-335) IHC-plus; WISP3; CCN family member 6; CCN6; PPD; LIBC; PPAC; WISP-3; Human WISP3; PPD

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

Polyclonal

Rabbit

Amino acids 320 to 335 of human WISP3

Protein G Purified

PBS, 0.1% sodium azide.

1 mg/ml (lot specific)

Long term: -20 degree C; Short term: +4 degree C. Avoid repeat 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.

Immunohistochemistry (IHC - Paraffin), ELISA (EIA)

ELISA (1:000 - 1:1000), IHC-P (10 ug/ml)
Usage: Immunohistochemistry: was validated for use in immunohistochemistry on a panel of 21 formalin-fixed, paraffin-embedded (FFPE) human tissues after heat induced antigen retrieval in pH 6.0 citrate buffer. After incubation with the primary antib...

Anti-WISP3 antibody IHC of human small intestine. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 10 ug/ml.

41,402 Da

WNT1 inducible signaling pathway protein 3

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

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; Secreted; Cell cycle regulation

Chromosomal Location of Human Ortholog: 6q21

Cellular Component: proteinaceous extracellular matrix; extracellular space

Molecular Function: integrin binding; heparin 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

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