Novel membrane cell projection defects in Wiskott-Aldrich syndrome B cells

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Novel membrane cell projection defects in Wiskott-Aldrich syndrome B cells.

Wiskott-Aldrich syndrome (WAS) is an X-linked immunodeficiency characterized by microthrombocytopenia, eczema, recurrent infections, autoimmune disorders and an increased incidence of malignancies. This complex phenotype results from mutations in the WASP gene. WASP is a key member of a protein family that links signaling pathways to actin cytoskeleton reorganization by activating Arp2/3-mediat...

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Defects in Wiskott-Aldrich syndrome blood cells.

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Age-Dependent Defects of Regulatory B Cells in Wiskott-Aldrich Syndrome Gene Knockout Mice

The Wiskott-Aldrich syndrome (WAS) is a rare X-linked primary immunodeficiency characterized by recurrent infections, thrombocytopenia, eczema, and high incidence of malignancy and autoimmunity. The cellular mechanisms underlying autoimmune complications in WAS have been extensively studied; however, they remain incompletely defined. We investigated the characteristics of IL-10-producing CD19+C...

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[The Wiskott-Aldrich syndrome].

can occur, the observed improvement cannot necessarily b)e attributed to the transfer factor. However, in two patients repeated remissions consistently followed transfer factor administration on repeated occasions. This included freedom from infections, regression of splenomegaly, and clearing of eczema. An unexpected finding was a decrease in bleeding in 3 of the 10 patients who had bleeding. ...

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Two novel mutations identified in the Wiskott-Aldrich syndrome protein gene cause Wiskott-Aldrich syndrome and thrombocytopenia.

Wiskott-Aldrich syndrome (WAS) and X-linked thrombocytopenia (XLT) are rare X-linked genetic disorders caused by mutations of the Wiskott-Aldrich syndrome protein (WASP) gene. Both disorders are clinically characterized by chronic thrombocytopenia of small platelets. WAS is a more severe form of the disorder and also courses with eczema, and immune dysfunction. In the present study, we investig...

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ژورنال

عنوان ژورنال: International Journal of Molecular Medicine

سال: 2007

ISSN: 1107-3756,1791-244X

DOI: 10.3892/ijmm.20.4.445