Various ASBT inhibitors such as Volixibat, Odevixibat, Maralixibat, Lopixibat, Elobixibat, Linerixibat, A4250, and GSK2333072 have entered clinical trials of which Maralixibat and Odevixibat have been approved for commercial development in the EU and USA for the treatment of Alagille’s disease and progressive familial intrahepatic cholestasis (PFIC) [172]. The gene discussed is SLC10A2; the disease is progressive familial intrahepatic cholestasis.