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Article

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

Proceedings of the National Academy of Sciences of the United States of America - 15 Nov 2011

Van Goor Fredrick, Hadida Sabine, Grootenhuis Peter D J, Burton Bill, Stack Jeffrey H, Straley Kimberly S, Decker Caroline J, Miller Mark, McCartney Jason, Olson Eric R, Wine Jeffrey J, Frizzell Ray A, Ashlock Melissa, Negulescu Paul A

Abstract excerpt

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene that impair the function of CFTR, an epithelial chloride channel required for proper function of the lung, pancreas, and other organs. Most patients with CF carry the F508del CFTR mutation, which...

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Identifiers

PMID: 21976485
DOI: 10.1073/pnas.1105787108
PMCID: PMC3219147

Genes

CFTR

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-21976485-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

Related research

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Research trail→

Click a neighbor to travelStep 1 · 12 closest

Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.PMID 21976485Read ↗

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 ↗

Corrector VX-809 stabilizes the first transmembrane domain of CFTR.Semantic proximity Read ↗

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.Semantic proximity Read ↗

Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression.Semantic proximity Read ↗

Pharmacological Correction of Cystic Fibrosis: Molecular Mechanisms at the Plasma Membrane to Augment Mutant CFTR Function.Semantic proximity Read ↗

Probing conformational rescue induced by a chemical corrector of F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutant.Semantic proximity Read ↗

Protein Processing and Inflammatory Signaling in Cystic Fibrosis: Challenges and Therapeutic StrategiesSemantic proximity Read ↗

Mechanistic Approaches to Improve Correction of the Most Common Disease-Causing Mutation in Cystic Fibrosis.Semantic proximity Read ↗

Regulation of CFTR Biogenesis by the Proteostatic Network and Pharmacological Modulators.Semantic proximity Read ↗

Cystic fibrosis transmembrane conductance regulator protein repair as a therapeutic strategy in cystic fibrosis.Semantic proximity Read ↗

Insights into the mechanisms underlying CFTR channel activity, the molecular basis for cystic fibrosis and strategies for therapy.Semantic proximity Read ↗

Increased efficacy of VX ‐809 in different cellular systems results from an early stabilization effect of F508del‐ CFTRSemantic proximity Read ↗

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