Congenital Hepatic Fibrosis (IMAGE) Tokyo Medical and Dental University Caption Congenital hepatic fibrosis (CHF) is a genetic liver disease due to the dysfunction of fibrocystin. In this study, genome-edited human iPS cells are utilized as a disease model of CHF. Fibrocystin dysfunction promotes the proliferation of cholangiocytes in an interleukin-8 (IL-8)-dependent manner, resulting in the bile duct malformation. IL-8 induces progressive liver fibrosis via the promotion of connective tissue growth factor (CTGF) production from cholangiocytes. These pathophysiological mechanisms of CHF are quite different from liver cirrhosis due to chronic hepatitis. Credit Department of Liver Disease Control TMDU Usage Restrictions None License Licensed content Disclaimer: AAAS and EurekAlert! are not responsible for the accuracy of news releases posted to EurekAlert! by contributing institutions or for the use of any information through the EurekAlert system.