The development of correctors and potentiators of the cystic fibrosis transmembrane conductance regulator (CFTR, ABCC7, OMIM: 602421) and the rescue of mutants of ABCB4, defective in progressive familial intrahepatic cholestasis type 3 (PFIC3, OMIM: 602347), suggest these or perhaps other small molecule chaperones may facilitate maturation and rescue function of G5G8 [146,147]. Here, ABCB4 is linked to intrahepatic cholestasis.