Article
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.
Scientific reports - 12 Apr 2022
da Cunha Mélanie Faria, Pranke Iwona, Sassi Ali, Schreiweis Christiane, Moriceau Stéphanie, Vidovic Dragana, Hatton Aurélie, Carlon Mariane Sylvia, Creste Geordie, Berhal Farouk, Prestat Guillaume, Freund Romain, Odolczyk Norbert, Jais Jean Philippe, Gravier-Pelletier Christine, Zielenkiewicz Piotr, Jullien Vincent, Hinzpeter Alexandre, Oury Franck, Edelman Aleksander, Sermet-Gaudelus Isabelle
Abstract excerpt
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's epithelium and the production of a thickened mucus favoring chronic bacterial colonization, sustained inflammation and...
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