Nv. Henriquez et al., ANTIGEN RECEPTOR-MEDIATED TRANSMEMBRANE SIGNALING IN WISKOTT-ALDRICH SYNDROME, The Journal of immunology, 153(1), 1994, pp. 395-399
The X-linked immunodeficiency Wiskott-Aldrich syndrome (WAS) is a cond
ition that includes a deficient anti-polysaccharide Ab response. Recen
tly, it has been suggested that B cells from patients with WAS show a
defective calcium mobilization response upon engagement of sIgM. Becau
se primarily EBV-transformed cells were used in these studies, we test
ed freshly isolated blood B cells for their calcium mobilization capab
ility upon engagement of sIg and CD19. No significant differences in t
he calcium mobilization capability of CD20(+) B cells of four individu
al WAS patients compared with capability in normal controls were found
. Receptor desensitization as assessed by calcium mobilization inhibit
ion also seemed to be intact. T cells were tested for their anti-CD3-i
nduced calcium flux and, again, no abnormalities could be observed whe
n compared with T cells from healthy individuals. We conclude that WAS
B and T cells can be stimulated into a normal calcium mobilization re
sponse 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.