FIG. 1.

(A) Hepatic FXR expression and MC presence increase in human liver disease explant patients. Livers from nondiseased control, PSC, PSC-IBD, PBC, biliary atresia, and NASH were stained using immunohistochemistry for FXR (brown), costained with tryptase (red) to detect activated MC presence. Enhanced biliary FXR immunoreactivity (marked by black arrows) is present in all diseased tissue explants compared with control, with a subset (PSC, PSC-IBD, and PBC) demonstrating increased hepatocyte FXR expression, indicating activated hepatic FXR function. Tryptase expression also increased in all models of disease compared with control. (B) Patients with PSC (n = 14) and PSC-IBD (n = 17) have increased serum FGF19 compared with healthy controls (n = 5). (C) Patients with PSC (n = 3) and PSC-IBD (n = 4) also have increased hepatic SHP expression compared with controls (n = 5). Data are mean ± SEM of n = 2 (serum) or n = 3 (qPCR) experiments per patient sample. *P < 0.05 vs. control. Representative images are presented as ×20 and zoom boxes are ×80.