Newer variants of progressive familial intrahepatic cholestasis

Figure 1 Pathogenesis of progressive familial intrahepatic cholestasis 1, 2 and 3. Familial intrahepatic cholestasis protein 1 is a flippase that helps in movement of phosphatidylserine and phosphatidylethanolamine from the outer to inner leaflet of the plasma membrane of hepatocyte; Bile salt exporter pump exports bile acid from hepatocytes to bile canaliculus; Multidrug resistance protein 3 is a floppase involved in transporting phosphatidylcholine into bile canaliculus. PFIC: Progressive familial intrahepatic cholestasis; FIC1: Familial intrahepatic cholestasis protein 1; BSEP: Bile salt exporter pump; MDR3: Multidrug resistance protein 3.