Hepatic Canalicular Membrane Transport of Bile Salt in C57L/J and AKR/J Mice: Implications for Cholesterol Gallstone Formation

Atp8b1 deficiency in mice reduces resist

Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport

✍ Coen C. Paulusma; Annemiek Groen; Cindy Kunne; Kam S. Ho-Mok; Astrid L. Spijkerb 📂 Article 📅 2006 🏛 John Wiley and Sons 🌐 English ⚖ 897 KB

Progressive familial intrahepatic cholestasis type 1 (PFIC1, Byler disease, OMIM 211600) is a severe inherited liver disease caused by mutations in ATP8B1. ATP8B1 is a member of the type 4 subfamily of P-type ATPases, which are phospholipid flippases. PFIC1 patients generally develop end-stage liver