BALB-Abcb4 mice lacking the hepatobiliary phospholipid transporter represent a potential model of hepatic osteodystrophy
K Hochrath, B Rathkolb, K Butuzova, W Hans, H Fuchs, V Gailus-Durner, E Wolf, M Hrabé de Angelis, F Lammert · Zeitschrift für Gastroenterologie · 2009 · Heft 09
Bibliografische Angaben
Zusammenfassung
Aims: Metabolic bone disease occurring in patients with chronic cholestasis, known as hepatic osteodystrophy, is a potential complication of long-standing liver diseases. Abcb4 (Mdr2) knockout mice develop chronic cholangitis due to the lack of phosphatidylcholine from bile. This mouse model resembles progressive familial intrahepatic cholestasis type 3 in humans, which is caused by mutations of the orthologous phosphatidylcholine transporter and may lead to cirrhosis in early childhood. In an attempt to characterize the systemic consequences of ABCB4 deficiency, we now phenotyped these mice in standardised, com…
