Synergy in B-Cell Activation between Toll-Like Receptor 9 and Transmembrane Activator and Calcium-Modulating Cyclophilin Ligand Interactor (TACI) in A181E/C104R Compound Heterozygous Siblings
Figure 2
TACI expression and function are decreased in the CVID patient compared to her asymptomatic sibling. (a) Pedigree of the studied family. Circles display females; squares display males. The index patient is indicated with an arrow. (b) Chest high-resolution CT scan of the index patient, revealing peribronchial consolidations with ground glass appearance and peribronchial thickening. ((c)-(d)) TACI surface (c ii) or intracellular (c iii) protein and mRNA expression. Light grey line indicated isotype indicated dark grey, TACI (c i). Results of 5 healthy donors: 3 CVID patients with normal TACI, 2 CVID patients with monoallelic A181E, the index patient and sibling 2. For (d) average results of 3 independent experiments are shown. (e) AID (left) and NFAT (right) mRNA induction after 48 h culture of LCL with the indicated stimuli. Bars represent means ± standard error of the mean.
