Comprehensive phenotypic characterization of ABCB4 deficient mice reveals systemic effects of spontaneous biliary fibrosis
K Hochrath, S Hillebrandt, F Lammert, B Rathkolb, H Fuchs, D Buch, T Adler, E Wolf, V Gailus-Durner, M Hrabé de Angelis · Zeitschrift für Gastroenterologie · 2009 · Heft 01
Bibliografische Angaben
Zusammenfassung
Aims: ABCB4 deficiency in humans causes or contributes to chronic cholestatic liver diseases. The progression of liver diseases related to ABCB4 deficiency and their systemic effects differ significantly between individuals, yet the genetic factors contributing to these mechanisms are unknown. Our aim now was to phenotypically characterize immune and extrahepatic phenotypes in a genetically defined model of spontaneous biliary fibrosis. Methods: We generated congenic mice by introgressing the Abcb4 knockout from the FVB/NJ strain into the fibrosis-susceptible BALB/cJ background for 10 generation. At the age of 1…
