The clinical relevance of BSEP in bile salt secretion in man has also been demonstrated by several genetic traits, such as progressive familial intrahepatic cholestasis type 2 (PFIC2), benign recurrent intrahepatic cholestasis (BRIC), and intrahepatic cholestasis of pregnancy (ICP) (for review see [2,3]). This evidence concerns the gene ABCB11 and benign recurrent intrahepatic cholestasis.