Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Jan 2017

Mesbahi Myriam, Shteinberg Michal, Wilschanski Michael, Hatton Aurelie, Nguyen-Khoa Thao, Friedman Hannah, Cohen Michael, Escabasse Virginie, Le Bourgeois Muriel, Lucidi Vicenzina, Sermet-Gaudelus Isabelle, Bassinet Laurence, Livnat Galit

Abstract excerpt

Ivacaftor, a CFTR potentiator, has been found to improve CFTR function and clinical outcomes in patients with cystic fibrosis (CF) gating mutations. We investigated the effects of ivacaftor on CFTR functional measurement in CF patients carrying gating mutations other than p.Gly551Asp. Two siblings aged 13 and 12 carrying the p.Ser549Asn mutation, two sisters (45 and 43years old) compound heterozygotes for...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Aminophenols
Share to Chloride Channel Agonists

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Chlorides

Share to Membrane Potentials

Share to Nasal Mucosa

Share to Outcome Assessment, Health Care

Share to Quinolones

Source

Identifiers

PMID: 27659740
DOI: 10.1016/j.jcf.2016.08.006

Genes

CFTR

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Changes of CFTR functional measurements and clinical improvements in cystic fibrosis patients with non p.Gly551Asp gating mutations treated with ivacaftor.PMID 27659740Read ↗

Effectiveness of ivacaftor in cystic fibrosis patients with non-G551D gating mutations.Semantic proximity Read ↗

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

Ivacaftor in a young boy with the rare gating mutation S549R--use of lung clearance index to track progress: a case report.Semantic proximity Read ↗

Sweat chloride as a biomarker of CFTR activity: proof of concept and ivacaftor clinical trial data.Semantic proximity Read ↗

Ivacaftor treatment of cystic fibrosis patients with the G551D mutation: a review of the evidence.Semantic proximity Read ↗

Normalization of sweat chloride concentration and clinical improvement with ivacaftor in a patient with cystic fibrosis with mutation S549N.Semantic proximity Read ↗

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

Ivacaftor in subjects with cystic fibrosis who are homozygous for the F508del-CFTR mutation.Semantic proximity Read ↗

Ivacaftor for patients with cystic fibrosisSemantic proximity Read ↗

Clinical Pharmacogenetics Implementation Consortium (CPIC) Guidelines for Ivacaftor Therapy in the Context of CFTR GenotypeSemantic proximity Read ↗

Ivacaftor for the p.Ser549Arg (S549R) gating mutation - The Israeli experience.Semantic proximity Read ↗

Efficacy and safety of ivacaftor in patients with cystic fibrosis who have an Arg117His-CFTR mutation: a double-blind, randomised controlled trial.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.