|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 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.
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) Cammer, M., et al. J. Biol. Chem. 284(35):23302-23311(2009)Zhang, J., et al. J. Biol. Chem. 284(32):21659-21669(2009)Ameratunga, R., et al. N. Z. Med. J. 122(1304):46-53(2009)
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