Evidence map›Paper›PMID 36117114›Full record

ReviewJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2023

Mucus aberrant properties in CF: Insights from cells and animal models.

Camille Ehre, Gunnar C Hansson, David J Thornton, Lynda S Ostedgaard

Open access · greenFull text readReview
In one paragraph

Review in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.

0numbers the graph read from it
0cells of the map it votes in
5citing papers in PubMed
1.1field-weighted citation impact, top 22% 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

5 citing papers in PubMed, 9 citations in OpenAlex.

  1. Review
  2. Article
  3. Cystic fibrosis.Nature reviews. Disease primers · 2024
    Review
  4. Review
  5. Membrane Contact Sites in Proteostasis and ER Stress Response.Contact (Thousand Oaks (Ventura County, Calif.))
    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

4 authors at 4 institutions in 3 countries.

Camille EhreUniversity of North Carolina at Chapel Hill, Department of Pediatrics, Marsico Lung Institute, Chapel Hill, NC, USA.
Gunnar C HanssonDepartment Medical Biochemistry and Cell Biology, University of Gothenburg, Gothenburg, Sweden.
David J ThorntonThe Wellcome Trust Centre for Cell-Matrix Research, and The Lydia Becker Institute of Immunology and Inflammation, Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
Lynda S OstedgaardDepartment of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA. Electronic address: lynda-ostedgaard@uiowa.edu.
University of Gothenburg · SEUniversity of Iowa · USUniversity of North Carolina at Chapel Hill · USWellcome Centre for Cell-Matrix Research · GB

Funding

Respiratory virus infection in early cystic fibrosis lung diseaseP01HL091842 · NHLBI · UNIVERSITY OF IOWA · PI ZABNER, JOSEPH · 2008 to 2022
$34.2M
Vector Core-Core 2P30DK054759 · NIDDK · UNIVERSITY OF IOWA · PI Alejandro Antonio Pezzulo · 1998 to 2026
$30.5M
Vector CoreP30DK065988 · NIDDK · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Scott H Randell · 2004 to 2026
$26.5M
Testing the Contributions of Airway Submucosal Glands and Surface Epithelia to Lung HealthR01HL163556 · NHLBI · UNIVERSITY OF IOWA · PI STOLTZ, DAVID A · 2022 to 2025
$2.5M
National Centre for the Replacement, Refinement and Reduction of Animals in Research NC/S001034/1NHLBI NIH HHS P01 HL091842NHLBI NIH HHS R01 HL163556NIDDK NIH HHS P30 DK054759NIDDK NIH HHS P30 DK065988
6 · The paper itself

Abstract

Cystic fibrosis (CF), an autosomal genetic disorder caused by the dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) protein, is characterized by mucus accumulation in the lungs, the intestinal tract, and the pancreatic ducts. Mucins are high-molecular-weight glycoproteins that govern the biochemical and biophysical properties of mucus. In the CF lung, increased mucus viscoelasticity is associated with decreased mucociliary clearance and defects in host defense mechanisms. The link between defective ion channel and abnormal mucus properties has been investigated in studies involving cell and animal models. In this review article, we discuss recent progress toward understanding the different regions and cells that express CFTR in the airways and how mucus is produced and cleared from the lungs. In addition, we reflect on animal models that provided insights into the organization and the role of the mucin network and how mucus and antimicrobial activities act in concert to protect the lungs from invading pathogens.

Indexed as

Cystic FibrosisAnimalsCystic Fibrosis Transmembrane Conductance RegulatorLungModels, AnimalMucinsMucusCystic Fibrosis Transmembrane Conductance RegulatorMucinsAirwayAnimal modelsCystic FibrosisMucusSubmucosal gland

Identifiers

PMID36117114
PMCPMC10018425
OpenAlexW4296050960

What OpenQuestion holds

Textfull text, public
LicenceCC BY
measurements read1
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.