ABCB11 and progressive familial intrahepatic cholestasis type 2: 4PBA is capable of restoring the cell surface expression of mislocated mutants on bile salt export pump (BSEP), which is related to an inherited autosomal recessive liver disease called progressive familial intrahepatic cholestasis type 2 (PFIC2) that leads to cirrhosis and death before adulthood [276].