Fig.2 Expression of IFNGR1 protein and phosphorylation of STAT1
in patient monocytes upon IFN-γ and IFN-α stimulation
Analysis of IFNGR1 (CD119, clone GIR-208) on patient CD45+CD3-CD14+
monocytes (blue) and healthy control (HC)(red). Patient 1 and Patient 2
showed reduced expression of IFN-γR1 in monocytes compared to the HC
(n=2) (A) Flowcytometric analysis of phosphorylated STAT1
(pSTAT1) protein in patient monocytes after stimulation with 60IU/ml
IFN-γ. Patient 1 had no pSTAT1 expression, Patient 2 had severely
reduced and Patient 3 had slightly reduced pSTAT1 expression. The median
fluorescence intensity (MFI) for two independent experiments are shown
in (B ) (HC n=2; Patient 1: n=2; Patient 2: n=2; Patient 3:
n=2). MFI of pSTAT1 for two independent experiments in patient monocytes
after stimulation with 10ˆ4IU/ml IFN-α. Patient 1 and 2 had normal
levels of pSTAT1, while the expression of pSTAT1 was slightly reduced in
Patient 3 after 5 minutes of stimulation. (HC n=2; Patient 1: n=1;
Patient 2: n=2; Patient 3: n=2) (C) Expression of SOCS1mRNA relative to GADPH in patient peripheral blood mononuclear
cells after stimulation with IFN-γ (HC: n= 2; Patients: n=2
respectively) (D ) or IFN-α (HC: n=1; Patient 1: n=1; Patient 2:
n=1; Patient 3: n=1) (E ) showed that Patient 1 and 2 have no
SOCS1 expression after IFN-γ stimulation, while stimulation with IFN-α
result in normal induction of SOCS1 expression. The expression ofSOCS1 is reduced in Patient 3 upon IFN-γ stimulation.
(F ) (HC: n=1; Patient 2: n=1. )