ArticleThe Journal of clinical investigation1997
Lung disease in mice with cystic fibrosis.
Article in The Journal of clinical investigation, 1997. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers.
What it found
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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.
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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.
Who cites it
51 citing papers in PubMed, 168 citations in OpenAlex.
- In Vivo Inflammation Caused byInternational journal of molecular sciences · 2023Article
- Acute polymicrobial airway infections: analysis in cystic fibrosis mice.Microbiology (Reading, England) · 2023Article
- Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis.Cellular and molecular life sciences : CMLS · 2021Review
- 2-O, 3-O desulfated heparin (ODSH) increases bacterial clearance and attenuates lung injury in cystic fibrosis by restoring HMGB1-compromised macrophage function.Molecular medicine (Cambridge, Mass.) · 2021Article
- Nanog maintains stemness of Lkb1-deficient lung adenocarcinoma and prevents gastric differentiation.EMBO molecular medicine · 2021Article
- Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene.JCI insight · 2021Article
- Article
- CFTR regulates B cell activation and lymphoid follicle development.Respiratory research · 2019Article
- AGTR2 absence or antagonism prevents cystic fibrosis pulmonary manifestations.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2019Article
- Chemically modified hCFTR mRNAs recuperate lung function in a mouse model of cystic fibrosis.Scientific reports · 2018Article
- Airway disease phenotypes in animal models of cystic fibrosis.Respiratory research · 2018Review
- Animal Models in the Pathophysiology of Cystic Fibrosis.Frontiers in pharmacology · 2018Review
- Partial Restoration of CFTR Function in cftr-Null Mice following Targeted Cell Replacement Therapy.Molecular therapy : the journal of the American Society of Gene Therapy · 2017Article
- Article
- Early pulmonary disease manifestations in cystic fibrosis mice.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2016Article
- Airway Hydration, Apical K(+) Secretion, and the Large-Conductance, Ca(2+)-activated and Voltage-dependent Potassium (BK) Channel.Annals of the American Thoracic Society · 2016Review
- Tracking the immunopathological response to Pseudomonas aeruginosa during respiratory infections.Scientific reports · 2016Article
- Ventilatory pattern and energy expenditure are altered in cystic fibrosis mice.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2013Article
- Loss of Cftr function exacerbates the phenotype of Na(+) hyperabsorption in murine airways.American journal of physiology. Lung cellular and molecular physiology · 2013Article
- Proteases, cystic fibrosis and the epithelial sodium channel (ENaC).Cell and tissue research · 2013Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
14 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have been compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite the presence of severe intestinal disease, to develop lung disease. Herein, we describe the phenotype of an inbred congenic strain of CFTR-knockout mouse that develops spontaneous and progressive lung disease of early onset. The major features of the lung disease include failure of effective mucociliary transport, postbronchiolar over inflation of alveoli and parenchymal interstitial thickening, with evidence of fibrosis and inflammatory cell recruitment. We speculate that the basis for development of lung disease in the congenic CFTR-knockout mice is their observed lack of a non-CFTR chloride channel normally found in CFTR-knockout mice of mixed genetic background.
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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.