Protein kinase a‐dependent pSer675‐β‐catenin, a novel signaling defect in a mouse model of congenital hepatic fibrosis
Carlo Spirli, Luigi Locatelli, Carola M. Morell, Romina Fiorotto, Stuart D. Morton, Massimiliano Cadamuro, Luca Fabris, Mario Strazzabosco – 6 June 2013 – Genetically determined loss of fibrocystin function causes congenital hepatic fibrosis (CHF), Caroli disease (CD), and autosomal recessive polycystic kidney disease (ARPKD). Cystic dysplasia of the intrahepatic bile ducts and progressive portal fibrosis characterize liver pathology in CHF/CD.