Intrahepatic bile acid retention in BALB/c-Abcb4-/--mice activates FXR-dependent rescue transporters at the basolateral and canalicular membrane

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive obliteration of bile ducts, accumulation of bile acids and secondary biliary fibrosis. Abcb4 involves the transport of phospholipids from hepatocytes into bile across the canalicular membrane. Abcb4 knockout disturbes the excretion of phospholipids into bile leading to a more toxic bile composition and bile duct alterations resemble sclerosing cholangitis. Hence we analyzed hepatic bile acid transport in Abcb4–/– mice during the course of the disease. Methods: Total serum bile acids were measured (TBA) by dia…