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Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome

Henriquez, NV; Rijkers, GT; Zegers, BJ; (1994) Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome. pp. 395-399.

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Type: Article
Title: Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome
Additional information: Henriquez, N V Rijkers, G T Zegers, B J United states Journal of immunology (Baltimore, Md. : 1950) J Immunol. 1994 Jul 1;153(1):395-9. The X-linked immunodeficiency Wiskott-Aldrich syndrome (WAS) is a condition that includes a deficient anti-polysaccharide Ab response. Recently, it has been suggested that B cells from patients with WAS show a defective calcium mobilization response upon engagement of sIgM. Because primarily EBV-transformed cells were used in these studies, we tested freshly isolated blood B cells for their calcium mobilization capability upon engagement of sIg and CD19. No significant differences in the calcium mobilization capability of CD20+ B cells of four individual WAS patients compared with capability in normal controls were found. Receptor desensitization as assessed by calcium mobilization inhibition also seemed to be intact. T cells were tested for their anti-CD3-induced calcium flux and, again, no abnormalities could be observed when compared with T cells from healthy individuals. We conclude that WAS B and T cells can be stimulated into a normal calcium mobilization response when their AgRs are cross-linked. It is highly improbable that the immune dysfunction observed in WAS patients is related to a direct disorder of their B and/or T cell AgRs.
Keywords: Adolescent Adult Antigens, CD/physiology Antigens, CD19 Antigens, Differentiation, B-Lymphocyte/physiology B-Lymphocytes/*physiology Calcium/metabolism Child Humans Lymphocyte Activation Receptors, Antigen, B-Cell/*physiology Receptors, Antigen, T-Cell/*physiology Receptors, Complement 3d/analysis Signal Transduction T-Lymphocyte Subsets/*physiology Wiskott-Aldrich Syndrome/*immunology
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Cancer Institute > Research Department of Pathology
URI: http://discovery.ucl.ac.uk/id/eprint/81594
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