Extrahepatic manifestations of ATP-binding cassette transporter B4 (ABCB4) deficiency detected in a systematic phenomics platform
K Hochrath, M Krawczyk, M Hrabĕ de Angelis, F Lammert · Zeitschrift für Gastroenterologie · 2014 · Heft 08
Bibliografische Angaben
Zusammenfassung
Background: The ABCB4 gene encodes the hepatocanalicular phospholipid floppase, which translocates phosphatidylcholine from liver into bile. Abcb4 knockout (Abcb4 -/-) mice develop chronic cholangitis and biliary fibrosis similar to primary sclerosing cholangitis in humans (Gastroenterology 2004;127:261 – 74). To date, the interactions of these diseases with extrahepatic organs are not fully defined. With the aim to characterize the full spectrum of systemic effects of ABCB4 deficiency, we performed comprehensive whole body phenomics of Abcb4 -/- mice. Methods: Standardized phenotyping screens (Nat Methods 2005;…