Reduced expression of BSEP at the canalicular membrane or impaired activity leads to accumulation of bile salts in the hepatocyte, which is a cause for benign recurrent or progressive familial intrahepatic cholestasis type 2 (BRIC2 and PFIC2, respectively) [5,6]. This evidence concerns the gene ABCB11 and progressive familial intrahepatic cholestasis type 2.