Wiskott-Aldrich Syndrome Protein (WAS, THC, IMD2, WASP, Eczema-thrombocytopenia)

The Wiskott-Aldrich syndrome (WAS) family of proteins share similar domain structure, and are involved in transduction of signals from receptors on the cell surface to the actin cytoskeleton. The presence of a number of different motifs suggests that they are regulated by a number of different stimuli, and interact with multiple proteins. Recent studies have demonstrated that these proteins, directly or indirectly, associate with the small GTPase, Cdc42, known to regulate formation of actin filaments, and the cytoskeletal organizing complex, Arp2/3. Wiskott-Aldrich syndrome is a rare, inherited, X-linked, recessive disease characterized by immune dysregulation and microthrombocytopenia, and is caused by mutations in the WAS gene. The WAS gene product is a cytoplasmic protein, expressed exclusively in hematopoietic cells, which show signalling and cytoskeletal abnormalities in WAS patients. These clinical findings as well as the structural properties of WASP (including a cdc42 binding site, SH3 domain binding region and regions for actin cytoskeletal localization) suggest a pivotal role for WASP in regulating the structure and function of platelets and T-lymphocytes. A transcript variant arising as a result of alternative promoter usage, and containing a different 5' UTR sequence, has been described, however, its full-length nature is not known. \n\nApplications:\nSuitable for use in Immunoblot and Immunoprecipitation. Other applications have not been tested.\n\nRecommended Dilution:\nImmunoblot Analysis: 2ug/ml detects WASP in RIPA lysates from human Jurkat and Raji and murine BAF-3 cells. Raji cell lysate was resolved by electrophoresis, transferred to nitrocellulose and probed with anti-Human WASP (2ug/ml). Proteins were visualized using a goat anti-rabbit secondary antibody conjugated to HRP and a chemiluminescence detection system.\n\nImmunoprecipitation: 4ug immunoprecipitates WASP from 500ug of human Jurkat and Raji and murine BAF-3 RIPA lysates. \n\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. For long-term storage , store at -20 degrees C. Stable for at least 12 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Prices direct from United States Biological

Quick response times

Exclusive Biosave savings/discounts

SPECIFICATIONS

Catalog Number

W0800

Size

200ug

Applications

IP, WB

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in 0.1M Tris-glycine, pH 7.4, 0.15M

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Stewart, D., et al., J. Clinical Invest. 97: 2627

Additional Info

Recognizes WASP (65kD) and an unknown 85kD protein in some cell lines. WASP has been described in literature as migrating on SDS-PAGE at an apparent MW ranging from 59-75kD. Crossreactivity with N-WASP, a homologous protein isolated from brain, has not been determined. Species crossreactivity: Human and mouse.