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Article

The Role of Ivacaftor in Severe Cystic Fibrosis in a Patient With the R117H Mutation.

Chest - 1 Sept 2015

Ronan Nicola J, Fleming Claire, O'Callaghan Grace, Maher Michael M, Murphy Desmond M, Plant Barry J

Abstract excerpt

Cystic fibrosis (CF) conductance transmembrane regulator functions as a chloride (Cl-) channel in multiple organs, including the lungs. More than 1,800 disease-associated mutations have been identified, which can be divided into six classes. In patients with CF due to class III gating mutations,...

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Identifiers

PMID: 26324139
DOI: 10.1378/chest.14-3215

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-26324139-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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The Role of Ivacaftor in Severe Cystic Fibrosis in a Patient With the R117H Mutation.PMID 26324139Read ↗

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Ivacaftor as salvage therapy in a patient with cystic fibrosis genotype F508del/R117H/IVS8-5T.Semantic proximity Read ↗

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Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.Semantic proximity Read ↗

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