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Antigen receptor-induced activation and cytoskeletal rearrangement are impaired in Wiskott-Aldrich syndrome protein-deficient lymphocytes

Author

András Nagy

1951 -

Josef Penninger

1964 -

John Mark Butler

Published in: Journal of Experimental Medicine

Date of publication: November 1, 1999

Language: English

Main subject: lymphocyte, cytoskeleton, Wiskott-Aldrich syndrome

Bibliographic databases:

Cites articles 70

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Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea
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Wiskott-Aldrich syndrome. An immunologic deficiency disease involving the afferent limb of immunity
October 1, 1969

Wiskott-Aldrich syndrome: qualitative platelet defects and short platelet survival
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Ligand-induced association of surface immunoglobulin with the detergent-insoluble cytoskeletal matrix of the B lymphocyte
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Cofilin, a protein in porcine brain that binds to actin filaments and inhibits their interactions with myosin and tropomyosin
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Newborn and Wiskott-Aldrich patient B cells can be activated by TNP-Brucella abortus: evidence that TNP-Brucella abortus behaves as a T-independent type 1 antigen in humans
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The Wiskott-Aldrich syndrome: studies of platelets, basophils and polymorphonuclear leucocytes
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Heterogeneity among T cells in intracellular free calcium responses after mitogen stimulation with PHA or anti-CD3. Simultaneous use of indo-1 and immunofluorescence with flow cytometry

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Novel blockade by brefeldin A of intracellular transport of secretory proteins in cultured rat hepatocytes
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Morphological abnormalities in the lymphocytes of patients with the Wiskott-Aldrich syndrome
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The increase of non-MHC-restricted cytotoxic cells (gamma/delta-TCR-bearing T cells or NK cells) and the abnormal differentiation of B cells in Wiskott-Aldrich syndrome
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Leu 23 induction as an early marker of functional CD3/T cell antigen receptor triggering. Requirement for receptor cross-linking, prolonged elevation of intracellular [Ca++] and stimulation of protein kinase C
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X-chromosome inactivation in the Wiskott-Aldrich syndrome: a marker for detection of the carrier state and identification of cell lineages expressing the gene defect
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Altered expression of leucocyte sialoglycoprotein in Wiskott-Aldrich syndrome is associated with a specific defect in O-glycosylation
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Neonatal lethality and lymphopenia in mice with a homozygous disruption of the c-abl proto-oncogene
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Altered O-glycan synthesis in lymphocytes from patients with Wiskott-Aldrich syndrome
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Aberrant O-linked oligosaccharide biosynthesis in lymphocytes and platelets from patients with the Wiskott-Aldrich syndrome
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Evidence for defective transmembrane signaling in B cells from patients with Wiskott-Aldrich syndrome
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T cells of patients with the Wiskott-Aldrich syndrome have a restricted defect in proliferative responses
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