Figure 7.

Flow cytometric assessment of pSTAT1 expression on monocytes. (A) Gating of monocytes on side scatter vs. CD14 FITC. (B) Overlay of patients with CR IFN-γR1 deficiencies showing an abolished response to IFN-γ. (C) Patients with CR IFN-γR1 showing a normal response to IFN-α compared with healthy controls, where green is patient unstained, red patient stained, and blue control stained. (D) Overlay of pSTAT1 expression in a healthy control and patient with PD IFN-γR1 deficiency showing an impaired response to increasing concentrations of IFN-γ (green is unstained, sky blue 10 IU/ml, violet 100 IU/ml, magenta 1,000 IU/ml, and red 10,000 IU/ml concentration of IFN-γ). (E) Baseline serum IFN-γ levels in patients with complete and partial IFN-γR1 deficiency, and controls (asterisks indicate statistical significance: **P < 0.0014).

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