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Article

Lumacaftor (VX-809) restores the ability of CF macrophages to phagocytose and kill Pseudomonas aeruginosa.

American journal of physiology. Lung cellular and molecular physiology - 1 Mar 2018

Barnaby Roxanna, Koeppen Katja, Nymon Amanda, Hampton Thomas H, Berwin Brent, Ashare Alix, Stanton Bruce A

Abstract excerpt

Cystic fibrosis (CF), the most common lethal genetic disease in Caucasians, is characterized by chronic bacterial lung infection and excessive inflammation, which lead to progressive loss of lung function and premature death. Although ivacaftor (VX-770) alone and ivacaftor in combination with lumacaftor (VX-809) improve lung function in CF patients with the Gly551Asp and del508Phe mutations, respectively, the...

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Identifiers

PMID: 29146575
DOI: 10.1152/ajplung.00461.2017
PMCID: PMC5900360

Genes

CFTR

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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