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

The Wiskott-Aldrich syndrome (WAS) is an X-chromosome-linked recessive disease characterized by eczema, thrombocytopenia, and immunodeficiency (1). WAS is an immunodeficiency disorder with the most severe pathology in the T lymphocytes and platelets. The disease arises from mutations in the gene encoding the WAS protein (WASP). T lymphocytes of affected males with WAS exhibit a severe disturbance of the actin cytoskeleton, suggesting that the WASP could regulate its organization. Data suggest that the WASP might function as a signal transduction adaptor downstream of Cdc42, and in affected males, the cytoskeletal abnormalities may result from a defect in Cdc42 signaling (2). WASP is a key regulator of the Arp2/3 complex and the actin cytoskeleton in hematopoietic cells. WASP is capable of forming an auto-inhibited conformation, which can be disrupted by binding of Cdc42 and phosphatidylinositol 4,5-bisphosphate, leading to its activation. Stimulation of the collagen receptor on platelets and crosslinking the B-cell receptor induce tyrosine phosphorylation of WASP (3).\n\nApplications:\nSuitable for use in Flow Cytometry, ELISA, Western Blot, Immunoprecipitation and Immunocytochemistry. Other applications not tested.\n\nRecommended Dilution:\nWestern Blot: 1:1000-1:2000\nImmunocytochemistry: 1:100-1:250\nFlow Cytometry: 1:30\nImmunoprecipitation: 1:30\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, aliquot and store at -20 degrees C. Aliquots are 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. \n\n\nManufactured incorporating RabMAb(R) technology under Epitomics US patents, No 5,675,063 and 7,429,487, owned by Abcam.

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SPECIFICATIONS

Catalog Number

W0807

Size

100ul

Applications

FC, ICC, IP, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a liquid in 50 mM Tris- Glycine, pH 7.4, 0.15 M sodium chloride, 40% Glycerol, 0.01% sodium azide and 0.05% BSA.

P Type

Mab

Purity

Supernatant

Isotype

IgG

References

1.Smith FJ, et al. Biochem Biophys Res Commun. 297(4):818-27, 2002.2.Adley BP, et al. Anal Quant Cytol Histol 28(4):228-36, 20063.Coons SW, et al. Endocr Pathol 16(3):201-10, 2005

Additional Info

Recognizes human WASP. Species crossreactivity: mouse and rat.