Genetic analysis of non-toxic liver fibrosis in a congenic mouse intercross

Background: Mutations in the ABCB4 (ATP-binding casette, subfamily B, member 4) gene cause cholestatic liver diseases including progressive intrahepatic familial cholestasis (PFIC). Modifier genes of these diseases have yet to be identified systematically. In this study we availed of the Abcb4 (Mdr2) knockout (-/-) mouse model, in which deficiency of the hepatobiliary phosphatidylcholine floppase leads to chronic cholestasis and fibrosis. As different mouse strains show variable fibrosis susceptibility, we applied a systematic approach to elucidate the genetic control of liver fibrosis in an experimental cross o…