De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: A novel mechanism of cholestasis
Verena Keitel, Martin Burdelski, Zsuzsanna Vojnisek, Lutz Schmitt, Dieter Häussinger, Ralf Kubitz – 29 July 2009 – Progressive familial intrahepatic cholestasis type 2 (PFIC‐2) is caused by mutations of the bile salt export pump (BSEP [ABCB11]), an ATP‐binding cassette (ABC)‐transporter exclusively expressed at the canalicular membrane of hepatocytes. An absence of BSEP from the canalicular membrane causes cholestasis and leads to liver cirrhosis, which may necessitate liver transplantation in early childhood.