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

Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Mar 2018

Kim Jeeyeon, Davies Zoe, Dunn Colleen, Wine Jeffrey J, Milla Carlos

Abstract excerpt

BACKGROUND: To determine in vivo effects of CFTR modulators on mutation S945L. METHODS: We measured effects of CFTR modulators on CFTR-dependent sweating ('C-sweat') in two pancreatic sufficient cystic fibrosis (CF) subjects. S1 (S945L/G542X) took ivacaftor and S2 (S945L/F508del) took ivacaftor+tezacaftor. Sweating was stimulated pharmacologically to produce sequentially both CFTR-independent (methacholine...

Read the complete abstract on PubMed

Topics

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

Share to Alleles
Share to Aminophenols

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Benzodioxoles

Share to Chloride Channel Agonists

Share to Cystic Fibrosis

Share to Cystic Fibrosis Transmembrane Conductance Regulator

Share to Indoles

Share to Quinolones

Source

Identifiers

PMID: 29279204
DOI: 10.1016/j.jcf.2017.12.005

Genes

CFTR

Variants

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.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.PMID 29279204Read ↗

The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurements.Semantic proximity Read ↗

In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands.Semantic proximity Read ↗

Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.Semantic proximity Read ↗

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

Real-world data confirm elexacftor/tezacaftor/ivacaftor modulators halves sweat chloride concentration in eligible people with cystic fibrosis.Semantic proximity Read ↗

Characterizing CFTR modulated sweat chloride response across the cf population: Initial results from the CHEC-SC study.Semantic proximity Read ↗

Acute administration of ivacaftor to people with cystic fibrosis and a G551D-CFTR mutation reveals smooth muscle abnormalitiesSemantic proximity Read ↗

Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis.Semantic proximity Read ↗

Effect of elexacaftor/tezacaftor/ivacaftor on mucus and mucociliary clearance in cystic fibrosis.Semantic proximity Read ↗

Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor.Semantic proximity Read ↗

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation.Semantic proximity Read ↗

How the sweat gland reveals levels of CFTR activity.Semantic proximity Read ↗

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