Mutations in the ATP8B1 are responsible for two related liver diseases, known as benign recurrent intrahepatic cholestasis type 1 (BRIC1) and the more severe form progressive familial intrahepatic cholestasis type 1 (PFIC1) (Bull et al., 1998). Here, ATP8B1 is linked to benign recurrent intrahepatic cholestasis type 1.