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Article

Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

American journal of respiratory and critical care medicine - 15 Jul 2014

Rowe Steven M, Heltshe Sonya L, Gonska Tanja, Donaldson Scott H, Borowitz Drucy, Gelfond Daniel, Sagel Scott D, Khan Umer, Mayer-Hamblett Nicole, Van Dalfsen Jill M, Joseloff Elizabeth, Ramsey Bonnie W

Abstract excerpt

RATIONALE: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator recently approved for patients with CF age 6 and older with the G551D mutation. OBJECTIVES: To evaluate ivacaftor in a postapproval setting and determine mechanism of action and response of clinically relevant markers. METHODS: We conducted a longitudinal cohort study in 2012-2013 in G551D CF patients age 6 and older...

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Identifiers

PMID: 24927234
DOI: 10.1164/rccm.201404-0703OC
PMCID: PMC4226057

Genes

CFTR

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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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