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Article

Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).

British journal of pharmacology - 1 Feb 2016

Matthes Elizabeth, Goepp Julie, Carlile Graeme W, Luo Yishan, Dejgaard Kurt, Billet Arnaud, Robert Renaud, Thomas David Y, Hanrahan John W

Abstract excerpt

BACKGROUND AND PURPOSE: The most common cystic fibrosis (CF) mutation F508del inhibits the gating and surface expression of CFTR, a plasma membrane anion channel. Optimal pharmacotherapies will probably require both a 'potentiator' to increase channel open probability and a 'corrector' that improves folding and trafficking of the mutant protein and its stability at the cell surface. Interaction between CF drugs...

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Identifiers

PMID: 26492939
DOI: 10.1111/bph.13365
PMCID: PMC4728415

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

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Low free drug concentration prevents inhibition of F508del CFTR functional expression by the potentiator VX-770 (ivacaftor).PMID 26492939Read ↗

Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosisSemantic proximity Read ↗

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.Semantic proximity Read ↗

The rescue of F508del-CFTR by elexacaftor/tezacaftor/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor.Semantic proximity Read ↗

Barriers to the Pharmacologic Rescue of W1282X CFTR.Semantic proximity Read ↗

Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.Semantic proximity Read ↗

Lipophilicity of the Cystic Fibrosis Drug, Ivacaftor (VX-770), and Its Destabilizing Effect on the Major CF-causing Mutation: F508del.Semantic proximity Read ↗

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Potentiator VX-770 (Ivacaftor) Opens the Defective Channel Gate of Mutant CFTR in a Phosphorylation-dependent but ATP-independent MannerSemantic proximity Read ↗

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.Semantic proximity Read ↗

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Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.Semantic proximity Read ↗

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