N-WASP Anticuerpo(C-1): sc-271484

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The Wiskott-Aldrich syndrome (WAS) is characterized by thrombocytopenia, eczema, defects in cell-mediated and humoral immunity, and a propensity for lymphoproliferative diseases. The syndrome is the result of a mutation in the gene encoding a proline-rich protein termed WASP. WASP and the related protein neural-WASP (or N-WASP) are downstream effectors of Cdc42. Both WASP and N-WASP are implicated in Actin polymerization and cytoskeletal organization, and N-WASP is also essential for mediating the Cdc42-induced formation of filopodia. WASP is primarily expressed in hematopoietic cells, whereas N-WASP is richest in neural tissues and is also expressed ubiquitously. The effects of Cdc42-stimulated Actin assembly require the interaction of WASP/N-WASP with the Arp2/3 complex, which dramatically enhances polymerization. The WASP and N-WASP proteins characteristically contain a pleckstrin homology (PH) domain, which binds phosphatidyl-inositol bisphosphate (PIP2); a Cdc42-binding domain; and a 70 amino acid conserved verprolin-homology (VPH) domain, which is the Actin-binding region and is critical to the regulation of the Actin cytoskeleton.