title: Antigen receptor-mediated transmembrane signaling in Wiskott-Aldrich syndrome.
abstract: 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.
authors.affiliation: Department of Immunology, University Hospital for Children and Youth, Utrecht, The Netherlands.
authors.forename: N V
authors.initials: NV
authors.lastname: Henriquez
authors.forename: G T
authors.initials: GT
authors.lastname: Rijkers
authors.forename: B J
authors.initials: BJ
authors.lastname: Zegers
mesh_terms: D000293:Adolescent; D000328:Adult; D015703:Antigens, CD / Q000502:physiology; D018941:Antigens, CD19; D000944:Antigens, Differentiation, B-Lymphocyte / Q000502:physiology; D001402:B-Lymphocytes / Q000502:physiology*; D002118:Calcium / Q000378:metabolism; D002648:Child; D006801:Humans; D008213:Lymphocyte Activation; D011947:Receptors, Antigen, B-Cell / Q000502:physiology*; D011948:Receptors, Antigen, T-Cell / Q000502:physiology*; D017464:Receptors, Complement 3d / Q000032:analysis; D015398:Signal Transduction; D016176:T-Lymphocyte Subsets / Q000502:physiology*; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology*
chemical_list: D015703:Antigens, CD; D018941:Antigens, CD19; D000944:Antigens, Differentiation, B-Lymphocyte; D011947:Receptors, Antigen, B-Cell; D011948:Receptors, Antigen, T-Cell; D017464:Receptors, Complement 3d; D002118:Calcium