Discovery and structural development of small molecules that enhance transport activity of bile salt export pump mutant associated with progressive familial intrahepatic cholestasis type 2

Highly E297G BSEP function enhancers, including 3-{2-Chloro-{4-[3-(2,6-dichlorophenyl)-5-isopropylisoxazol-4-yl]methoxy}-N-benzylbenzamido}benzoic acid, were discovered.