Abstract Background/Aims: Multidrug resistance protein 2 (Abcb4) gene knockout mice
(Mdr2−/−) lack phosphatidylcholine (PC) excretion into bile and spontaneously develop …

Abstract Multidrug resistance 2 (Mdr2), also called adenosine triphosphate-binding cassette
B4 (ABCB4), is the transporter of phosphatidylcholine (PC) at the canalicular membrane of …

Background & Aims: mdr2 P-glycoprotein (Pgp) deficiency in mice leads to the absence of
biliary phospholipids and cholesterol in the presence of normal bile salt secretion. The aim …

Background/Aims: The mouse mdr2 gene encodes a P-glycoprotein expressed in the
canalicular membrane of the hepatocyte. Mice in which this gene has been inactivated …

Bile acid imbalance causes progressive familial intrahepatic cholestasis type 2 (PFIC2) or
type 3 (PFIC3), severe liver diseases associated with genetic defects in the biliary bile acid …

Aim and Methods: A decreased function of multidrug‐resistance 3 P‐glycoprotein (MDR3),
limiting the rate of biliary phospholipid secretion, predisposes individuals to cholestasis …

Background & Aims: Because the mechanisms leading to bile duct damage in sclerosing
cholangitis are unknown, we aimed to determine the pathogenesis of bile duct injury in …

Deficiency of multidrug resistance 2 (mdr2), a canalicular phospholipid floppase, leads to
excretion of low‐phospholipid “toxic” bile causing progressive cholestasis. We hypothesize …

Mutations in multidrug resistance 3 gene (MDR3 or ABCB4) underlie progressive familial
intrahepatic cholestasis type 3 (PFIC3), a severe pediatric liver disease progressing to …

Background & Aims: It has been proposed that biliary phospholipids fulfill specific functions
in the absorption of dietary fat from the intestine, but the physiological significance has not …