ABCB4 and familial intrahepatic cholestasis: Mice deficient in the canalicular phospholipid flippase (Mdr2/Abcb4-/- mice) spontaneously develop liver injury (PSC; Shimamura et al., 2008), (Fichtner-Feigl et al., 2006; Jinnin et al., 2006; Fickert et al., 2009; Baghdasaryan et al., 2010; Mair et al., 2010; McHedlidze et al., 2013), which resembles PSC and most closely mimics MDR3 deficiency in patients,(Pope et al., 2005) which ranges from familial intrahepatic cholestasis to adult liver cirrhosis.