Normal responses to dsRNA, dsDNA, and ssRNA introduced by transfection in the patients’ fibroblasts. (a) Production of IFN-β and IFN-λ in response to various doses of poly(I:C), introduced by transfection into control (C+; averaged from two different control cell lines), P1, P2, and UNC-93B– and NEMO-deficient fibroblasts. Values represent mean values ± SD calculated from three independent experiments. (b) Production of IFN-β and IFN-λ in response to various doses of poly(dA:dT) introduced by transfection into control (C+), P1, P2, UNC-93B^−/−, and NEMO^−/− fibroblasts. Values from three different experiments were averaged and are presented here as mean values ± SD. (c) IFN-β production 24 or 48 h after transfection with the ssRNA 7sk-as in control (C+1 and C+2), P1, TLR3^−/−, and NEMO^−/− fibroblasts. Values represent mean values ± SD from three independent experiments. (d) IRF3 dimerization, assessed by native Western blotting upon stimulation with 2.25 ng/µl 7sk-as in fibroblasts from P1, at 8 and 12 h; comparison with control WT cells (C+), TLR3^−/−, and NEMO^−/− fibroblasts. The blots shown are representative of three independent experiments. NS, nonstimulated.