ArticleJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society2016
Early pulmonary disease manifestations in cystic fibrosis mice.
Article in Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 2016. 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.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 33 citations in OpenAlex.
- The Effects of Thirdhand Vape Residue from Nicotine and Non-Nicotine Vapes on Cells: A Systematic Review.International journal of environmental research and public health · 2025Pooled it
- Assessment of respiratory mechanics and X-ray velocimetry functional imaging in two cystic fibrosis rat models.Scientific reports · 2024Article
- Adjuvant effect of inhaled particulate matter containing free radicals following house-dust mite induction of asthma in mice.Inhalation toxicology · 2023Article
- GAA deficiency disrupts distal airway cells in Pompe disease.American journal of physiology. Lung cellular and molecular physiology · 2023Article
- Club cell CREB regulates the goblet cell transcriptional network and pro-mucin effects of IL-1B.Frontiers in physiology · 2023Article
- Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis.Cell reports · 2022Article
- The circadian system in cystic fibrosis mice is regulated by histone deacetylase 6.American journal of physiology. Cell physiology · 2022Article
- Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles.The journal of physiological sciences : JPS · 2022Article
- Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.Scientific reports · 2021Article
- Oscillometry of the respiratory system: a translational opportunity not to be missed.American journal of physiology. Lung cellular and molecular physiology · 2021Review
- MCTR1 Intervention Reverses Experimental Lung Fibrosis in Mice.Journal of inflammation research · 2021Article
- Airway Epithelial Telomere Dysfunction Drives Remodeling Similar to Chronic Lung Allograft Dysfunction.American journal of respiratory cell and molecular biology · 2020Article
- Electronic-Cigarette Vehicles and Flavoring Affect Lung Function and Immune Responses in a Murine Model.International journal of molecular sciences · 2020Article
- Dysregulation of Circadian Rhythm Gene Expression in Cystic Fibrosis Mice.Journal of circadian rhythms · 2019Article
- AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Small adipose stores in cystic fibrosis mice are characterized by reduced cell volume, not cell number.American journal of physiology. Gastrointestinal and liver physiology · 2018Article
- Review
- Airway disease phenotypes in animal models of cystic fibrosis.Respiratory research · 2018Review
- Strategies for the etiological therapy of cystic fibrosis.Cell death and differentiation · 2017Review
- Posttreatment with Protectin DX ameliorates bleomycin-induced pulmonary fibrosis and lung dysfunction in mice.Scientific reports · 2017Article
Corrections and comments
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Authors and funding
9 authors at 1 institution in 1 country.
Funding
Abstract
backgroundAltered pulmonary function is present early in the course of cystic fibrosis (CF), independent of documented infections or onset of pulmonary symptoms. New initiatives in clinical care are focusing on detection and characterization of preclinical disease. Thus, animal models are needed which recapitulate the pulmonary phenotype characteristic of early stage CF.
methodsWe investigated young CF mice to determine if they exhibit pulmonary pathophysiology consistent with the early CF lung phenotype. Lung histology and pulmonary mechanics were examined in 12- to 16-week-old congenic C57bl/6 F508del and R117H CF mice using a forced oscillation technique (flexiVent).
resultsThere were no significant differences in the resistance of the large airways. However, in both CF mouse models, prominent differences in the mechanical properties of the peripheral lung compartment were identified including decreased static lung compliance, increased elastance and increased tissue damping. CF mice also had distal airspace enlargement with significantly increased mean linear intercept distances.
conclusionsAn impaired ability to stretch and expand the peripheral lung compartment, as well as increased distances between gas exchange surfaces, were present in young CF mice carrying two independent Cftr mutations. This altered pulmonary histopathophysiology in the peripheral lung compartment, which develops in the absence of infection, is similar to the early lung phenotype of CF patients.
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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.