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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 ABCB11 gene cause a spectrum of rare liver diseases. The most severe form is progressive familial intrahepatic cholestasis type 2 (PFIC2). Current medical treatments have limited effic...

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Detalles Bibliográficos
Autores principales: Mareux, Elodie, Lapalus, Martine, Ben Saad, Amel, Zelli, Renaud, Lakli, Mounia, Riahi, Yosra, Almes, Marion, Banet, Manon, Callebaut, Isabelle, Decout, Jean-Luc, Falguières, Thomas, Jacquemin, Emmanuel, Gonzales, Emmanuel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9502978/
https://www.ncbi.nlm.nih.gov/pubmed/36142670
http://dx.doi.org/10.3390/ijms231810758