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Article

Ivacaftor treatment in patients with cystic fibrosis and the G551D-CFTR mutation.

European respiratory review : an official journal of the European Respiratory Society - 1 Mar 2013

Sermet-Gaudelus Isabelle

Abstract excerpt

Cystic fibrosis (CF) is an autosomal recessive lethal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene that encodes for CFTR, an epithelial cell-surface expressed protein responsible for the transport of chloride (Cl(-)). Gating mutations associated with defective conductance can be modulated by CFTR potentiators. Ivacaftor is a CFTR potentiator approved for the...

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Source

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Identifiers

PMID: 23457167
DOI: 10.1183/09059180.00008512
PMCID: PMC9487423

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.

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