Wiskott–Aldrich syndrome
Evidence for defective transmembrane signaling in B cells from patients with Wiskott Aldrich syndrome
... disorder, Wiskott-Aldrich syndrome (WAS), fail to produce antibodies in response to stimulation with polysaccharides and other type-2 T cell-independent ...
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Neural Wiskott Aldrich syndrome protein modulates Wnt signaling and is required for hair follicle cycling in mice
... function. Wiskott-Aldrich syndrome family proteins act downstream of these GTPases, control- ling actin assembly and cytoskeletal reorganization, but their role in epithelial cells has not been ...
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Wiskott Aldrich syndrome protein is required for regulatory T cell homeostasis
... Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency disorder characterized by opportunistic, viral, and bacterial infections due to abnormal lymphocyte ...
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A Novel Site Mutation in A Wiskott-Aldrich Syndrome Boy: A Case Report
... Wiskott-Aldrich syndrome (WAS) is a sort of X-linked rare disorder which dominantly affects male ...by Wiskott in 1937, and then in 1954 Aldrich et ...
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Hypercatabolism of IgG, IgA, IgM, and albumin in the Wiskott Aldrich syndrome: A unique disorder of serum protein metabolism
... The Wiskott-Aldrich syndrome is an immune deficiency disorder with an impairment of both humoral and cellular immune ...the Wiskott-Aldrich syndrome using purified radioiodinated ...
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Second site mutation in the Wiskott Aldrich syndrome (WAS) protein gene causes somatic mosaicism in two WAS siblings
... Revertant mosaicism due to true back mutations or second-site mutations has been identified in several inherited disorders. The occurrence of revertants is considered rare, and the underlying genetic mechanisms remain ...
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Mycolactone activation of Wiskott Aldrich syndrome proteins underpins Buruli ulcer formation
... identify Wiskott-Aldrich syndrome protein (WASP) and neural WASP (N-WASP) as molecular targets of ...complex syndrome of immune defects, whereas N-WASP is more widely expressed ...
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Polarized Hyphal Growth in Candida albicans Requires the Wiskott-Aldrich Syndrome Protein Homolog Wal1p
... The yeast-to-hypha transition is a key feature in the cell biology of the dimorphic human fungal pathogen Candida albicans. Reorganization of the actin cytoskeleton is required for this dimorphic switch in Candida. We ...
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Abnormalities of Chemotactic Lymphokine Synthesis and Mononuclear Leukocyte Chemotaxis in Wiskott Aldrich Syndrome
... from Wiskott-Aldrich syndrome patients was impaired, particularly in those patients with the highest rates of unstimulated LDCF ...from Wiskott-Aldrich syndrome ...in ...
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Dendritic cell defects in Wiskott-Aldrich Syndrome
... m ild WAS but missense mutations elsewhere and other types o f mutations, found predominantly in exons 6-11, give rise to a range o f illness severity (Derry et al, 1995; Zhu et al, 1997; Lemahieu et al, 1999), making ...
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Identification and characterisation of the interaction between Wiskott-Aldrich syndrome protein (WASP) and c-Src kinases
... this syndrome was ...for Wiskott-Aldrich Syndrome Protein, and mutations in the WASP gene were found to be responsible for classical WAS and attenuated forms of WAS, including isolated ...
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Lentiviral mediated gene therapy restores B cell tolerance in Wiskott Aldrich syndrome patients
... patients irrespectively of age and disease severity scores (14, 17, 18). Human WASp-deficient memory B cells showed a preferential usage of variable Ig heavy chain VH4-34, a gene segment encod- ing intrinsically ...
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Nuclear Wiskott–Aldrich syndrome protein co-regulates T cell factor 1-mediated transcription in T cells
... WASp is uniquely expressed in hematopoietic cells and its role for their normal function have been exten- sively studied in Wiskott–Aldrich syndrome (WAS) pa- tients that lack expression of WASp and ...
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Clinical and Molecular Characters of a Yemeni Child with Wiskott Aldrich Syndrome
... with Wiskott-Aldrich Syndrome. Wiskott-Aldrich Syndrome is a rare condition which needs a high index of suspicion for early di- agnosis and should be suspected in any male infant ...
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The Novel Adaptor Protein, Mti1p, and Vrp1p, a Homolog of Wiskott-Aldrich Syndrome Protein-Interacting Protein (WIP), May Antagonistically Regulate Type I Myosins in Saccharomyces cerevisiae
... Type I myosins in yeast, Myo3p and Myo5p (Myo3/5p), are involved in the reorganization of the actin cytoskeleton. The SH3 domain of Myo5p regulates the polymerization of actin through interactions with both Las17p, a ...
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Studies of the expression of the Wiskott Aldrich syndrome protein
... The Wiskott-Aldrich syndrome (WAS) is an X-linked disor- der characterized by thrombocytopenia, eczema, disorders in cell-mediated and humoral immunity, and a proclivity to lymphoproliferative ...
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The Wiskott Aldrich Syndrome: RESULTS OF TRANSFER FACTOR THERAPY
... 12 patients with Wiskott-Aldrich syndrome were treated with therapeutic doses of transfer factor in an attempt to induce cellular immunity. Clinical improvement was noted after transfer factor ...
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Intermittent low platelet counts hampering diagnosis of X-linked thrombocytopenia in children: report of two unrelated cases and a novel mutation in the gene coding for the Wiskott-Aldrich syndrome protein
... Cases presentation: Both cases described were boys who presented with an intermittent pattern of thrombocytopenia, with a persistently low mean platelet volume. In both patients, peripheral blood smear revealed small ...
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Autoimmunity in Wiskott-Aldrich Syndrome: Risk Factors, Clinical Features, and Outcome in a Single-Center Cohort of 55 Patients
... arthritis, autoimmunity, autoimmune hemolytic anemia, children, hematopoietic stem cell transplantation, immuno- deficiency, thrombocytopenia, vasculitis, Wiskott-Aldrich syndrome.. ABBR[r] ...
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Wiskott-Aldrich Syndrome Protein Is Needed for Vaccinia Virus Pathogenesis
... Although smallpox has been eradicated, little is known of the mechanisms responsible for the high mortality rate due to this disease (9, 21). Since vaccination has been discontinued (9), recent acts of bioterrorism and ...
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