ArticleJCI insight2016
Acute administration of ivacaftor to people with cystic fibrosis and a
Article in JCI insight, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 40 citations in OpenAlex.
- Considerations for early life genetic therapies in cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2026Review
- Potentiation of BKCa channels by cystic fibrosis transmembrane conductance regulator correctors VX-445 and VX-121.The Journal of clinical investigation · 2024Article
- Pulmonary Vascular Dysfunctions in Cystic Fibrosis.Physiology (Bethesda, Md.) · 2024Review
- Subclinical vascular, hemodynamic and arterial stiffness changes in adults with cystic fibrosis: cross-sectional observational study.Scientific reports · 2024Observational
- CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity.Respiratory research · 2023Article
- Infection, Allergy, and Inflammation: The Role ofMicroorganisms · 2023Review
- Pharmacologic improvement of CFTR function rapidly decreases sputum pathogen density, but lung infections generally persist.The Journal of clinical investigation · 2023Observational
- Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond.The European respiratory journal · 2023Review
- Patient and Caregiver Perceptions of Airway Clearance Methods Used for Cystic Fibrosis.Canadian respiratory journal · 2023Article
- Prevalence and Determinants of Wheezing and Bronchodilatation in Children With Cystic Fibrosis: A Retrospective Cohort Study.Frontiers in pediatrics · 2022Article
- Review
- Inflammatory cytokines TNF-α and IL-17 enhance the efficacy of cystic fibrosis transmembrane conductance regulator modulators.The Journal of clinical investigation · 2021Article
- Amphotericin B induces epithelial voltage responses in people with cystic fibrosis.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2021Article
- Asthma in Cystic Fibrosis: Definitions and Implications of This Overlap Syndrome.Current allergy and asthma reports · 2021Review
- Subacute TGFβ Exposure Drives Airway Hyperresponsiveness in Cystic Fibrosis Mice through the PI3K Pathway.American journal of respiratory cell and molecular biology · 2020Article
- Ivacaftor decreases monocyte sensitivity to interferon-γ in people with cystic fibrosis.ERJ open research · 2020Article
- CFTR Modulator Use Is Associated with Higher Hemoglobin Levels in Individuals with Cystic Fibrosis.Annals of the American Thoracic Society · 2019Article
- The Strain on Airway Smooth Muscle During a Deep Inspiration to Total Lung Capacity.Journal of engineering and science in medical diagnostics and therapy · 2019Article
- CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine.Frontiers in pharmacology · 2019Review
- Acquired Cystic Fibrosis Transmembrane Conductance Regulator Dysfunction and Radiographic Bronchiectasis in Current and Former Smokers: A Cross-Sectional Study.Annals of the American Thoracic Society · 2019Article
Corrections and comments
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Authors and funding
16 authors at 6 institutions in 3 countries.
Funding
Abstract
backgroundAirflow obstruction is common in cystic fibrosis (CF), yet the underlying pathogenesis remains incompletely understood. People with CF often exhibit airway hyperresponsiveness, CF transmembrane conductance regulator (CFTR) is present in airway smooth muscle (ASM), and ASM from newborn CF pigs has increased contractile tone, suggesting that loss of CFTR causes a primary defect in ASM function. We hypothesized that restoring CFTR activity would decrease smooth muscle tone in people with CF.
methodsTo increase or potentiate CFTR function, we administered ivacaftor to 12 adults with CF with the
resultsIvacaftor rapidly restored CFTR function, indicated by reduced sweat chloride concentration. Airflow obstruction and air trapping also improved. Airway distensibility increased in airways less than 4.5 mm but not in larger-sized airways. To assess smooth muscle function in a tissue outside the lung, we measured vascular pulse wave velocity (PWV) and augmentation index, which both decreased following CFTR potentiation. Finally, change in distensibility of <4.5-mm airways correlated with changes in PWV.
conclusionsAcute CFTR potentiation provided a unique opportunity to investigate CFTR-dependent mechanisms of CF pathogenesis. The rapid effects of ivacaftor on airway distensibility and vascular tone suggest that CFTR dysfunction may directly cause increased smooth muscle tone in people with CF and that ivacaftor may relax smooth muscle.
fundingThis work was funded in part from an unrestricted grant from the Vertex Investigator-Initiated Studies Program.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.