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Article

CFTR correctors potentiate gating mutants causing cystic fibrosis.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2026

Castanier Solène, Elbahnsi Ahmad, Chevalier Benoit, Berri Lynda, Dias Wickramanayaka Madara, Mornon Jean-Paul, Hatton Aurélie, Sermet-Gaudelus Isabelle, Edelman Aleksander, Callebaut Isabelle, Hinzpeter Alexandre

Abstract excerpt

The Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulator VX-445 (Elexacaftor) used to treat cystic fibrosis presents both corrector and potentiator activities. This drug binds to a pocket within the CFTR membrane-spanning domain (MSD) assembly, in contact with the lasso motif. We have previously shown that the corrector activity of VX-445 is modulated by mutations within MSD1 and the nucleotide...

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Identifiers

PMID: 41266217
DOI: 10.1016/j.jcf.2025.11.008

Genes

CFTR

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

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