Finally, Ivacaftor has also shown therapeutic potential in an in vitro model of progressive familial intrahepatic cholestasis type 2 (PFIC2) caused by ABCB11 missense mutations affecting bile salt export pump (BSEP): Ivacaftor treatment increased the taurocholate transport activity of mutated BSEP by 1.7-fold, reaching 95% of BSEP function [60]. This evidence concerns the gene ABCB11 and progressive familial intrahepatic cholestasis type 2.