Functional Defect of Variants in the Adenosine Triphosphate-Binding Sites of ABCB4 and Their Rescue by the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator, Ivacaftor ( VX-770)-MedSci.cn

Functional Defect of Variants in the Adenosine Triphosphate-Binding Sites of ABCB4 and Their Rescue by the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator, Ivacaftor ( VX-770)

Delaunay, JL; Bruneau, A; Hoffmann, B; Anne-Marie; Durand-Schneider; Barbu, V; Jacquemin, E; Maurice, M; Housset, C; Callebaut, I; Ait-Slimane, T

Ait-Slimane, T (reprint author), Fac Med Pierre & Marie Curie, St Antoine Res Ctr, UPMC INSERM, 27 rue Chaligny, F-75571 Paris, France.

HEPATOLOGY, 2017; 65 (2): 560

Abstract

ABCB4 (MDR3) is an adenosine triphosphate (ATP)-binding cassette (ABC) transporter expressed at the canalicular membrane of hepatocytes, where it medi......

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Functional Defect of Variants in the Adenosine Triphosphate-Binding Sites of ABCB4 and Their Rescue by the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator, Ivacaftor ( VX-770)

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