Evidence map›Paper›PMID 37003609›Full record

ReviewThe European respiratory journal2023

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond.

Marcus A Mall, Gerard J Criner, Marc Miravitlles, Steven M Rowe, Claus F Vogelmeier, David J Rowlands, Matthias Schoenberger, Pablo Altman

Open access · greenAbstract readReview
In one paragraph

Review in The European respiratory journal, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed, 1 pooled it
6.4field-weighted citation impact, top 2% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

25 citing papers in PubMed, 1 synthesis or guideline pooled it, 44 citations in OpenAlex.

  1. Guideline
  2. Trial
  3. Sweat components as a promising monitoring tool for systemic diseases.The journal of physiological sciences : JPS · 2026
    Review
  4. Review
  5. Review
  6. Article
  7. Article
  8. Article
  9. Article
  10. A translational view of airway epithelial dysfunction in COPD.European respiratory review : an official journal of the European Respiratory Society · 2025
    Review
  11. Article
  12. Article
  13. Article
  14. Article
  15. Article
  16. Article
  17. Article
  18. Article
  19. Neutrophil serine proteases in cystic fibrosis: role in disease pathogenesis and rationale as a therapeutic target.European respiratory review : an official journal of the European Respiratory Society · 2024
    Review
  20. New drugs, new challenges in cystic fibrosis care.European respiratory review : an official journal of the European Respiratory Society · 2024
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors at 6 institutions in 4 countries.

Marcus A MallDepartment of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité-Universitätsmedizin Berlin, Berlin, Germany marcus.mall@charite.de.ORCID 0000-0002-4057-2199
Gerard J CrinerDepartment of Thoracic Medicine and Surgery, Lewis Katz School of Medicine at Temple University, Philadelphia, PA, USA.
Marc MiravitllesPneumology Department Hospital Universitari Vall d'Hebron, Vall d'Hebron Institut de Recerca (VHIR), Vall d'Hebron Hospital Campus, CIBER de Enfermedades Respiratorias (CIBERES), Barcelona, Spain.ORCID 0000-0002-9850-9520
Steven M RoweUniveristy of Alabama at Birmingham, Birmingham, AL, USA.ORCID 0000-0001-9045-0133
Claus F VogelmeierDepartment of Medicine, Pulmonary and Critical Care Medicine, University of Marburg, member of the German Center for Lung Research (DZL), Marburg, Germany.
David J RowlandsNovartis Institutes for Biomedical Research, Cambridge, MA, USA.
Matthias SchoenbergerNovartis Pharma AG, Basel, Switzerland.
Pablo AltmanNovartis Pharmaceuticals Corporation, East Hanover, NJ, USA.
German Center for Lung Research · DENovartis (United States) · USCentro de Investigación Biomédica en Red de Enfermedades Respiratorias · ESNovartis (Switzerland) · CHTemple University · USUniversity of Alabama at Birmingham · US

Funding

UAB CF Research and Translation Core CenterP30DK072482 · NIDDK · UNIVERSITY OF ALABAMA AT BIRMINGHAM · PI AMIT GAGGAR · 2007 to 2026
$23.0M
NIDDK NIH HHS P30 DK072482
6 · The paper itself

Abstract

The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified in a broad range of cell types including epithelial, endothelial, immune and structural cells. While CFTR has been investigated largely in the context of inborn dysfunction in cystic fibrosis, recent evidence shows that CFTR is also affected by acquired dysfunction in COPD. In patients with COPD and smokers, CFTR impairment has been demonstrated in the upper and lower airways, sweat glands and intestines, suggesting both pulmonary and systemic defects. Cigarette smoke, a key factor in COPD development, is the major cause of acquired CFTR dysfunction. Inflammation, bacterial byproducts and reactive oxygen species can further impair CFTR expression and function. CFTR dysfunction could contribute directly to disease manifestation and progression of COPD including disturbed airway surface liquid homeostasis, airway mucus obstruction, pathogen colonisation and inflammation. Mucus plugging and neutrophilic inflammation contribute to tissue destruction, development of dysfunction at the level of the small airways and COPD progression. Acquired CFTR dysfunction in extrapulmonary organs could add to common comorbidities and the disease burden. This review explores how CFTR dysfunction may be acquired and its potential effects on patients with COPD, particularly those with chronic bronchitis. The development of CFTR potentiators and the probable benefits of CFTR potentiation to improve tissue homeostasis, reduce inflammation, improve host defence and potentially reduce remodelling in the lungs will be discussed.

Indexed as

Cystic Fibrosis Transmembrane Conductance RegulatorPulmonary Disease, Chronic ObstructiveHumansInflammationLungRespiratory MucosaCystic Fibrosis Transmembrane Conductance Regulator

Identifiers

PMID37003609
PMCPMC10066568
OpenAlexW4311881860

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
Read underepoch 390

Registered trials

None linked

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.