Hyper-IgM syndrome type 4 with a B lymphocyte–intrinsic selective deficiency in Ig class-switch recombination
J. Clin. Invest. Kohsuke Imai, et al. 112:136 doi:10.1172/JCI18161 [
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Figure 2B cell responses to sCD40L+rIL-4 activation. (
a) Normal B cell proliferative response to sCD40L+rIL-4 stimulation in HIGM4 patients. PBMCs from age-matched controls (
n = 24), HIGM2 patients (
n = 16), and HIGM4 patients (
n = 13) were stimulated with sCD40L and rIL-4 for 5 days. White bars, no stimulation; black bars, 12-day stimulation with sCD40L+IL-4. Mean uptakes of [
3H]thymidine (cpm) are shown. Error bars show the SD. (
b) Defective IgE and IgG CSR following stimulation by sCD40L+rIL-4 in HIGM4 patients. PBMCs from normal controls (
n = 20 for IgE,
n = 5 for IgG), HIGM2 patients (
n = 20), and HIGM4 patients (
n = 13 for IgE,
n = 4 for IgG) were stimulated with sCD40L+rIL-4 for 12 days. White bars, no stimulation; black bars, 12-day stimulation with sCD40L+IL-4. Concentrations of IgE and IgG in the culture supernatants were quantified by ELISA. Mean values are shown. Error bars show the SD.
