|Other Names||Wiskott-Aldrich syndrome protein, WASp, WAS, IMD2|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Effector protein for Rho-type GTPases. Regulates actin filament reorganization via its interaction with the Arp2/3 complex. Important for efficient actin polymerization. Possible regulator of lymphocyte and platelet function. Mediates actin filament reorganization and the formation of actin pedestals upon infection by pathogenic bacteria.|
|Cellular Location||Cytoplasm, cytoskeleton.|
|Tissue Location||Expressed predominantly in the thymus. Also found, to a much lesser extent, in the spleen|
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The Wiskott-Aldrich syndrome (WAS) family of proteinsshare similar domain structure, and are involved in transduction ofsignals from receptors on the cell surface to the actincytoskeleton. The presence of a number of different motifssuggests that they are regulated by a number of different stimuli,and interact with multiple proteins. Recent studies havedemonstrated that these proteins, directly or indirectly, associatewith the small GTPase, Cdc42, known to regulate formation of actinfilaments, and the cytoskeletal organizing complex, Arp2/3.Wiskott-Aldrich syndrome is a rare, inherited, X-linked, recessivedisease characterized by immune dysregulation andmicrothrombocytopenia, and is caused by mutations in the WAS gene.The WAS gene product is a cytoplasmic protein, expressedexclusively in hematopoietic cells, which show signalling andcytoskeletal abnormalities in WAS patients. A transcript variantarising as a result of alternative promoter usage, and containing adifferent 5' UTR sequence, has been described, however, itsfull-length nature is not known.
Burns, S.O., et al. Blood 115(26):5355-5365(2010)Taylor, M.D., et al. Sci Transl Med 2 (37), 37RA44 (2010) :Rajmohan, R., et al. FEMS Yeast Res. 9(8):1226-1235(2009)Dovas, A., et al. J. Cell. Sci. 122 (PT 21), 3873-3882 (2009) :Ameratunga, R., et al. N. Z. Med. J. 122(1304):46-53(2009)
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