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Ivacaftor-Mediated Potentiation of ABCB4 Missense Mutations Affecting Critical Motifs of the NBDs: Repositioning Perspectives for Hepatobiliary Diseases

ABCB4 (ATP-binding cassette subfamily B member 4) is a hepatocanalicular floppase involved in biliary phosphatidylcholine (PC) secretion. Variations in the ABCB4 gene give rise to several biliary diseases, including progressive familial intrahepatic cholestasis type 3 (PFIC3), an autosomal recessive...

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Detalles Bibliográficos
Autores principales:	Delaunay, Jean-Louis, Elbahnsi, Ahmad, Bruneau, Alix, Madry, Claire, Durand-Schneider, Anne-Marie, Stary, Anne, Housset, Chantal, Gautheron, Jérémie, Callebaut, Isabelle, Aït-Slimane, Tounsia
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2023
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9867378/ https://www.ncbi.nlm.nih.gov/pubmed/36674751 http://dx.doi.org/10.3390/ijms24021236

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