PMC6249401_01
(A) Analysis of IFN-gamma-induced STAT1 phosphorylation in leukocytes by flow cytometry. IFN-gamma (10 ng/mL) increased p-STAT1 in healthy controls but did not increase p-STAT1 in patient. Recombinant human IFN-gamma was added to Jurkat cells inoculated with serum. After cells were permeabilized, anti-pSTAT1 antibodies were conjugated, and cells were subsequently analysed by flow cytometry, revealing that anti-IFN-gamma autoantibodies worked as neutralizing antibodies in this patient. (B) MRCP showing intrahepatic bile duct dilation (white arrow). (C) Endoscopic retrograde cholangiopancreatography showing hilar bile duct stricture (black arrow). IFN-gamma, interferon gamma; MRCP, magnetic resonance cholangiopancreatography; p-STAT1, phosphorylated signal transducer and activator of transcription 1; STAT1, signal transducer and activator of transcription 1.
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These antibodies are also potent inhibitors of IFN-gamma-stimulated signal transducer and activator of transcription 1 (STAT1) phosphorylation in leukocytes using a previously described method (Fig. 1(A)). Contrast-enhanced abdominal computed tomography and magnetic resonance cholangiopancreatography (Fig. 1(B)) and hilar bile duct stricture by endoscopic retrograde cholangiopancreatography (ERCP) (Fig. 1(C)) revealed intrahepatic bile duct dilation.