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Article

Net benefit of ivacaftor during prolonged tezacaftor/elexacaftor exposure in vitro.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jul 2022

Shaughnessy Ciaran A, Zeitlin Pamela L, Bratcher Preston E

Abstract excerpt

BACKGROUND: A decrease in the lumacaftor-mediated increase in F508del-CFTR function and expression upon prolonged exposure to ivacaftor (VX-770) has previously been described. However, the efficacy observed with ivacaftor-containing CFTR modulator therapies in vivo is in conflict with these reports. We hypothesized that a portion of the apparent decrease in CFTR function observed after prolonged ivacaftor...

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Identifiers

PMID: 35248469
DOI: 10.1016/j.jcf.2022.02.011
PMCID: PMC9329187

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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.

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