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Article

Development, clinical utility, and place of ivacaftor in the treatment of cystic fibrosis.

Drug design, development and therapy - 1 Jan 2013

O'Reilly Ruth, Elphick Heather E

Abstract excerpt

Cystic fibrosis (CF) is a life-limiting, multisystem disease characterized by thick viscous secretions leading to recurrent lung infections, bronchiectasis, and progressive deterioration in lung function. CF is caused by loss or dysfunction of the CF transmembrane conductance regulator (CFTR) protein which is responsible for transepithelial chloride and water transport. Improved understanding of CFTR protein...

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Source

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Identifiers

PMID: 24039402
DOI: 10.2147/DDDT.S30345
PMCID: PMC3770629

Genes

CFTR

Variants

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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