Fig. 3.

Impaired cGAMP-induced responses and preserved TLR3 and TLR9 signaling in patient cells. PBMC from healthy controls and P1 (A–E), or his mother (F–J), were stimulated with 100 µg/mL 2′3′-cGAMP for 6 h. Total RNA was isolated and mRNA levels of IFNB, IFNA2, CXCL10, MX1, and TNFA were measured relative to TBP. K–M PBMCs from P1 and three healthy controls were stimulated with poly(I:C) (50 µg/mL) or CpG ODN2395 (5 µg/mL) for 6h, after which total RNA was isolated and levels of IFNB1, CXCL10, and MX1 were measured relative to 18S mRNA. Statistics: 2-way ANOVA with Sidak’s multiple comparisons test. Ns, non-significant; *p ≤ 0.05, **p < 0.01, ****p ≤ 0.0001