Progressive familial intrahepatic cholestasis (PFIC) is a severe inherited form of cholestasis. This disease consists of 3 types (PFIC1-3) and is caused by mutations in ATP8B1, ABCB11 and ABCB4 respectively. All these genes encode for transport proteins in the canalicular membrane of the hepatocyte that are involved in proper bile formation. In the past, mouse models for all 3 forms of PFIC were studied and revealed a mild phenotype compared to human patients, which is most likely the result of a difference in bile salt hydrophobicity between mice and humans. For her PhD dissertation, Cindy Kunne examines a new mouse model with reduced bile salt hydroxylation capacity in order to create a more human like mouse model for cholestasis.