Figure 2. AD-CMC cells or transfection of cells with the mutated STAT1 inhibited IL-12R- and IL-23R-induced genes, but increased IFN-γ signaling.
Defective IL-12R (A) and IL-23R (B) pathways in cells isolated from AD-CMC patients. Data represent mean + SEM from 3 healthy controls and 3 AD-CMC patients (P3 and P4 from family #1 and P3 from family 2). (C) Stimulation of the IFN-γ receptor pathway results in increased production of the proinflammatory cytokine TNFα. Data represent mean + SEM from 3 healthy controls and 3 AD-CMC patients. CD4+ T cells from healthy controls were transfected with wild-type or mutant STAT1 plasmid. (D) Decreased transcription of STAT4 upon stimulation with IL-1β (10 ng/mL) and IL-23 (50 ng/mL), (E) and decreased transcription of MCP1 upon IL-12 (10 ng/mL) and IL-18 (10 ng/mL) stimulation. (F) The CD4+ cells transfected with mutant STAT1 plasmid showed increased transcription of PD-L1 upon stimulation with IFN-γ (1 µg/mL). Data represent mean + SEM from 6 healthy controls from 3 separate experiments.