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

The European respiratory journal - 1 Feb 2022

Becq Frédéric, Mirval Sandra, Carrez Thomas, Lévêque Manuella, Billet Arnaud, Coraux Christelle, Sage Edouard, Cantereau Anne

Abstract excerpt

Trikafta, currently the leading therapeutic in cystic fibrosis (CF), has demonstrated a real clinical benefit. This treatment is the triple combination therapy of two folding correctors elexacaftor/tezacaftor (VX445/VX661) plus the gating potentiator ivacaftor (VX770). In this study, our aim was to compare the properties of F508del-CFTR in cells treated with either lumacaftor (VX809), tezacaftor, elexacaftor,...

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Identifiers

PMID: 34266939
DOI: 10.1183/13993003.00671-2021

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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The rescue of F508del-CFTR by elexacaftor/tezacaftor/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor.PMID 34266939Read ↗

The preclinical discovery and development of the combination of ivacaftor + tezacaftor used to treat cystic fibrosis.Semantic proximity Read ↗

Rescue of multiple class II CFTR mutations by elexacaftor+tezacaftor+ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator.Semantic proximity Read ↗

Chronic and acute modulator treatment restore wild-type-like activity and stability to the primary cystic fibrosis-causing CFTR variant.Semantic proximity Read ↗

Effect of elexacaftor and bamocaftor on the metabolic and thermal stability of the F508del-CFTR protein in human airway epithelial cells.Semantic proximity Read ↗

Pharmacological rescue of the G85E CFTR variant by preclinical and approved modulatorsSemantic proximity Read ↗

Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro.Semantic proximity Read ↗

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Organic Synthesis and Current Understanding of the Mechanisms of CFTR Modulator Drugs Ivacaftor, Tezacaftor, and Elexacaftor.Semantic proximity Read ↗

Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination.Semantic proximity Read ↗

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CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTR.Semantic proximity Read ↗

Beyond Kaftrio : mechanistic insights to maximize N1303K-CFTR rescueSemantic proximity Read ↗

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