Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice

Abstract Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator gene (CFTR) are responsible for Cystic Fibrosis (CF). The most common CF-causing mutation is the deletion of the 508th amino-acid of CFTR (F508del), leading to dysregulation of the epithelial fluid transport in the airway’...

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Main Authors: Mélanie Faria da Cunha, Iwona Pranke, Ali Sassi, Christiane Schreiweis, Stéphanie Moriceau, Dragana Vidovic, Aurélie Hatton, Mariane Sylvia Carlon, Geordie Creste, Farouk Berhal, Guillaume Prestat, Romain Freund, Norbert Odolczyk, Jean Philippe Jais, Christine Gravier-Pelletier, Piotr Zielenkiewicz, Vincent Jullien, Alexandre Hinzpeter, Franck Oury, Aleksander Edelman, Isabelle Sermet-Gaudelus
Format: Artigo
Jezik:Inglês
Izdano: Nature Portfolio 2022-04-01
Serija:Scientific Reports
Online dostop:https://doi.org/10.1038/s41598-022-09678-9
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