The Wiskott-Aldrich syndrome (WAS) is an X-linked (Xpll.22) recessive immunodeficiency syndrome characterized by susceptibility to opportunistic and pyogenic infections, thrombocytopenia, and eczema. Previous studies of obligate carriers of WAS documented that nonrandom inactivation of the X chromosome carrying the defective gene is observed in all peripheral blood cells. The existence of b...

An important signaling pathway to the actin cytoskeleton links the Rho family GTPase Cdc42 to the actin-nucleating Arp2/3 complex through N-WASP. Nevertheless, these previously identified components are not sufficient to mediate Cdc42-induced actin polymerization in a physiological context. In this paper, we describe the biochemical purification of Toca-1 (transducer of Cdc42-dependent actin as...

Arp2/3 complex is believed to induce de novo nucleation of actin filaments at the edge of motile cells downstream of WASp family proteins. In this study, the signaling pathways leading to Arp2/3 complex activation, actin assembly, and shape change were investigated in platelets isolated from patients with Wiskott-Aldrich Syndrome (WAS), that is, who lack WASp, and in WASp-deficient mouse platel...

In 1937 Wiskott originally described the combination of eczema, thrombocytopenia, and increased susceptibility to infection in 3 brothers. Aldrich, Steinberg, and Campbell (1954) subsequently characterized this syndrome as a sex-linked recessive disorder, describing a family which included 40 males, 16 of whom died in infancy, and 10 of whom were known to have had eczema, otitis, and bloody dia...

wiskott-aldrich is an x-lined recessive disorder typically characterized by thrombocytopenia, eczema and recurrent infections. we report the four year treatment progress of a six year old boy who initially presented with vesicular lesions over the trunk, upper and lower extremities and face and blood tinged stools at the age of 2 weeks. from the family pedigree, there were two suspected cases t...

In 1937 Wiskott originally described the combination of eczema, thrombocytopenia, and increased susceptibility to infection in 3 brothers. Aldrich, Steinberg, and Campbell (1954) subsequently characterized this syndrome as a sex-linked recessive disorder, describing a family which included 40 males, 16 of whom died in infancy, and 10 of whom were known to have had eczema, otitis, and bloody dia...