The cystic fibrosis transmembrane conductance regulator controls biliary epithelial inflammation and permeability by regulating Src tyrosine kinase activity

• Published in: Hepatology (Baltimore, Md.) Vol. 64; no. 6; pp. 2118 - 2134
• Main Authors: Fiorotto, Romina, Villani, Ambra, Kourtidis, Antonis, Scirpo, Roberto, Amenduni, Mariangela, Geibel, Peter J., Cadamuro, Massimiliano, Spirli, Carlo, Anastasiadis, Panos Z., Strazzabosco, Mario
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.12.2016
• Subjects: Animals; Bile Ducts - cytology; Bile Ducts - enzymology; Cell Membrane; Cells, Cultured; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator - physiology; Epithelium; Hepatology; Inflammation - etiology; Kinases; Liver diseases; Mice; Permeability; Rodents; src-Family Kinases - physiology; Toll-Like Receptor 4 - physiology

In the liver, the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) regulates bile secretion and other functions at the apical membrane of biliary epithelial cells (i.e., cholangiocytes). CF‐related liver disease is a major cause of death in patients with CF. CFTR dysfunction affects i...