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WASP (WAS, THC, IMD2, WASP, Eczema-thrombocytopenia)

Cat no: W0807-01


Supplier: United States Biological
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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). Applications: Suitable for use in Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested. Recommended Dilution: Western Blot: 1:10,000-50,000 Immunohistochemistry: 1:100-1:250 Immunocytochemistry: 1:100-1: 250 Optimal dilutions to be determined by the researcher. Storage and Stability: May 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 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Manufactured incorporating RabMAb(R) technology under Epitomics US patents, No 5,675,063 and 7,429,487, owned by Abcam.
Catalogue number: W0807-01
Reactivities: Human
Hosts: Rabbit
Applications: Immunocytochemistry, Immunohistochemistry, Western Blot
Size: 100ul
Form: Supplied as a liquid in 50mM Tris-Glycine, pH 7.4, 0.15M sodium chloride, 0.01% sodium azide, 0.05% BSA, 40% Glycerol.
P type: Mab
Isotype: IgG
Purity: Supernatant
References: 1. Smith FJ, et al. Biochem Biophys Res Commun. 297(4):818-27, 2002. 2 .Adley BP, et al. Anal Qnt Cytol Histol 28(4):228-36, 2006 3. Coons SW, et al. Endocr Pathol 16(3):201-10, 2005
Additional info: Recognizes human WASP.

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