ABCB11 and progressive familial intrahepatic cholestasis type 2: Indeed, disruption of BSEP expression, localization, or function will decrease BA efflux and bile flow, and promotes primary cholestatic disorders such as progressive familial intrahepatic cholestasis type 2 (PFIC2), intrahepatic cholestasis of pregnancy (ICP), and drug-induced cholestasis (2).