Compound of formula II ** Formula ** in which M is CH2 or NH； R1 is H or hydroxyl； R2 is H, CH3, -CH2CH3, -CH2CH2CH3, -CH2CH2CH2CH3, -CH (CH3) 2, -CH2CH (CH3) 2, -CH (CH3) CH2CH3, CH2OH, -CH2OCH3, -CH (OH) CH3, -CH2SCH3 or -CH2CH2SCH3； or a pharmaceutically acceptable salt thereof, for use in the prophylaxis and / or treatment of a liver disease selected from the group consisting of Alagilles syndrome (ALGS)； progressive familial intrahepatic cholestasis (PFIC)； primary biliary cirrhosis (PBC)； Primary sclerosing cholangitis (PSC) and pruritus of cholestatic liver disease.Compuesto de fórmula II**Fórmula** en la que M es CH2 o NH； R1 es H o hidroxilo； R2 es H, CH3, -CH2CH3, -CH2CH2CH3, -CH2CH2CH2CH3, -CH(CH3)2, -CH2CH(CH3)2, -CH(CH3)CH2CH3, CH2OH, -CH2OCH3, -CH(OH)CH3, -CH2SCH3 o -CH2CH2SCH3； o una sal farmacéuticamente aceptable del mismo, para su uso en la profilaxis y/o el tratamiento de una enfermedad hepática seleccionada del grupo que consiste en síndrome de Alagilles (ALGS)； colestasis intrahepática familiar progresiva (PFIC)； cirrosis biliar primaria (PBC)； colangitis esclerosante primaria (PSC) y prurito de enfermedad hepática colestásica.
