Modelling hepatic osteodystrophy: BALB-Abcb4 knockout mice lacking the hepatobiliary phospholipid transporter display decreased 25-OH-vitamin D3 serum levels and bone mineral cont…

Abcb4 knockout mice (Abcb4–/– ) develop chronic cholangitis and biliary fibrosis due to the deficiency of biliary phosphatidylcholine. Hepatic osteodystrophy (HO) is a generic definition for the metabolic bone disease that may occur in individuals with chronic liver disease, especially in cholestatic patients with abnormalities of calcium homeostasis and vitamin D metabolism. Two distinct bone metabolic processes, osteoporosis and osteomalacia are combined together in various proportions in HO syndromes. Hence, in an attempt to characterize the systemic consequences of ABCB4 deficiency on bone metabolism, we phe…