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Bile Salt Export Pump (BSEP) is a member of the ATP-binding cassette (ABC) transporters, which mediates the transport of bile acid, taurocholate and other cholate conjugates across the hepatocyte canalicular membrane into the canaliculus. BSEP is associated with progressive familial intrahepatic cholestasis type 2 (PFIC2) and benign recurrent intrahepatic cholestasis type 2 (BRIC2). PFIC2 caused by mutations in the BSEP gene increases the risk of hepatocellular carcinoma in early life.