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. 2011 Sep 26;208(10):2083–2098. doi: 10.1084/jem.20101568

Figure 3.

Figure 3.

Absence of response to TLR3 in the patient’s fibroblasts. (A) Production of IFN-λ, IFN-β, and IL-6 by SV40-fibroblasts after stimulation with various doses of poly(I:C) for 12 or 24 h, as assessed by ELISA, with cells from a healthy control (C), the patient (P), a patient with partial AD TLR3 deficiency (AD TLR3), a patient with complete AR UNC-93B deficiency (UNC-93B−/−), and a NEMO-deficient patient (NEMO IP). The panels illustrate mean values ± SD for triplicates of one experiment, representative of three performed. (B) TLR3 mRNA levels in SV40-fibroblasts were determined by RT-qPCR on RNA samples from five healthy controls (C), the patient (P), an AD TLR3 patient, and a UNC-93B−/− patient. β-Glucuronidase (GUS) was used for normalization. One representative experiment of three performed is shown. (C) IL29 (IFN-λ1) and IFNB mRNA levels in SV40-fibroblasts from a control, the patient, an AD TLR3 patient, and a UNC-93B−/− patient, unstimulated (NS) and stimulated for 2, 4, 6, and 8 h with poly(I:C). The panels illustrate results from a single experiment, representative of three performed. (D) Production of IFN-λ and IL-6 by SV40-fibroblasts from a control, the patient, an AD TLR3 patient, a UNC-93B−/− patient, and a NEMO IP patient, unstimulated or after stimulation with the TLR3-specific agonist poly(A:U) for 12 and 24 h, as assessed by ELISA. The panels illustrate mean values ± SD for triplicates of one experiment, representative of three performed. (E) IRF-3 monomers and dimers in total cell extracts of SV40-fibroblasts from a control, the patient, an AD TLR3 patient, and a UNC-93B−/− patient, after stimulation with poly(I:C) for 1 and 2 h, as assessed by Western blotting. The results shown are representative of three independent experiments. (F) NF-κB activation was assessed by monitoring expression of the NF-κB luciferase reporter in SV40-fibroblasts from a control, the patient, an AD TLR3 patient, a UNC-93B−/− patient, and a NEMO IP patient, unstimulated or after stimulation with poly(I:C) (top) and IL-1β (bottom) for 6 h. The panels illustrate mean values ± SD for three independent experiments.