WASP (Wiskott-Aldrich Syndrome Protein, WAS, IMD2, 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 gene product is a cytoplasmic protein, expressed exclusively in hematopoietic cells, which show signalling and cytoskeletal abnormalities in WAS patients. 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 ELISA and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nELISA: 1:128,000\nWestern Blot: 0.03-0.1ug/ml, observed in human lysates of Tcell line MOLT4 and monocytic cell line U937 on ~60kD bands\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

W0800-0K

Size

100ug

Applications

ELISA, WB

Hosts

Goat

Reactivities

Hum, Mouse, Rat, Can

Form

Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

References

1. Yamaguchi H, Lorenz M, Kempiak S, Sarmiento C, Coniglio S, Symons M, Segall J, Eddy R, Miki H, Takenawa T, Condeelis J. Molecular mechanisms of invadopodium formation: the role of the N-WASP-Arp2/3 complex pathway and cofilin. J Cell Biol. 2005 Jan 31;168(3):441-52.

Additional Info

Recognizes human WAS. Species sequence homology: Canine, mouse and rat.