In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators

In Vitro Rescue of the Bile Acid Transport Function of ABCB11 Variants by CFTR Potentiators

ABCB11 is responsible for biliary bile acid secretion at the canalicular membrane of hepatocytes. Variations in the <i>ABCB11</i> gene cause a spectrum of rare liver diseases. The most severe form is progressive familial intrahepatic cholestasis type 2 (PFIC2). Current medical treatments...

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Bibliographic Details
Main Authors: Elodie Mareux, Martine Lapalus, Amel Ben Saad, Renaud Zelli, Mounia Lakli, Yosra Riahi, Marion Almes, Manon Banet, Isabelle Callebaut, Jean-Luc Decout, Thomas Falguières, Emmanuel Jacquemin, Emmanuel Gonzales
Format: Article
Language:English
Published: MDPI AG 2022-09-01
Series:International Journal of Molecular Sciences
Subjects:
BSEP
cholestasis
VX-770
potentiator
ABC transporter
pediatrics
Online Access:https://www.mdpi.com/1422-0067/23/18/10758

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https://www.mdpi.com/1422-0067/23/18/10758

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