Defective BCR- and TLR9-induced B-cell activation in CD19-deficient patients. A, Decreased CD19 cell surface expression in subjects carrying CD19 mutated allele(s). The cell surface expression of CD19 and CD20 was analyzed on CD20+ purified B cells from healthy family members (CD19-non carrier), CD19-heterozygous carriers (CD19-het.) and CD19-deficient patients (CD19-def.) of two unrelated families using flow cytometry. B, Altered TLR9 responses in B cells carrying CD19 mutated allele(s). Surface expression of TACI, CD23, CD80, CD86, CD69 and CD25 on CD20+CD27− naive B cells of a healthy family member (CD19-non carrier), a CD19-heterozygous carrier (CD19-het.) and a CD19-deficient patient (CD19-def.) after no stimulation or in vitro stimulation with F(ab′)2 anti-IgM or the TLR9 ligand CpG for two days. The frequency for the various B-cell surface markers after activation for two days as analyzed above is represented in C. Each symbol represents an individual, horizontal bars display the mean.