ArticleThe American journal of pathology2004
Characteristic multiorgan pathology of cystic fibrosis in a long-living cystic fibrosis transmembrane regulator knockout murine model.
Article in The American journal of pathology, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 70 papers, 1 of them a synthesis that pooled it.
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Who cites it
70 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Estrogen sulfotransferase in the metabolism of estrogenic drugs and in the pathogenesis of diseases.Expert opinion on drug metabolism & toxicology · 2019Pooled it
- Male reproductive phenotype in cystic fibrosis: comparison of existing animal models.Lab animal · 2026Review
- Quantifying abnormal alveolar microstructure in cystic fibrosis lung disease via hyperpolarizedJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2024Article
- Pathogenicity and virulence ofVirulence · 2023Review
- Cystic fibrosis rabbits develop spontaneous hepatobiliary lesions and CF-associated liver disease (CFLD)-like phenotypes.PNAS nexus · 2023Article
- Animal models of cystic fibrosis in the era of highly effective modulator therapies.Current opinion in pharmacology · 2022Review
- Current clinical opinion on CFTR dysfunction and patient risk of pancreatitis: diagnostic and therapeutic considerations.Expert review of gastroenterology & hepatology · 2022Article
- Selected transgenic murine models of human autoimmune liver diseases.Pharmacological reports : PR · 2022Review
- Methodological tools to study species of the genus Burkholderia.Applied microbiology and biotechnology · 2021Review
- Gene therapy for cystic fibrosis: new tools for precision medicine.Journal of translational medicine · 2021Review
- Rodent models of cholestatic liver disease: A practical guide for translational research.Liver international : official journal of the International Association for the Study of the Liver · 2021Review
- Tissue Engineering in Liver Regenerative Medicine: Insights into Novel Translational Technologies.Cells · 2020Review
- Animal models for cystic fibrosis liver disease (CFLD).Biochimica et biophysica acta. Molecular basis of disease · 2019Review
- Non-obstructive vas deferens and epididymis loss in cystic fibrosis rats.Mechanisms of development · 2019Article
- Diet-Induced Dysbiosis and Genetic Background Synergize With Cystic Fibrosis Transmembrane Conductance Regulator Deficiency to Promote Cholangiopathy in Mice.Hepatology communications · 2018Article
- Cholangiopathies - Towards a molecular understanding.EBioMedicine · 2018Review
- Ductal Mucus Obstruction and Reduced Fluid Secretion Are Early Defects in Chronic Pancreatitis.Frontiers in physiology · 2018Article
- Animal Models in the Pathophysiology of Cystic Fibrosis.Frontiers in pharmacology · 2018Review
- Article
- Increased susceptibility of Cftr-/- mice to LPS-induced lung remodeling.American journal of physiology. Lung cellular and molecular physiology · 2016Article
10 more citing papers are in PubMed but not listed here.
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Authors and funding
4 authors.
Funding
Abstract
The lack of an appropriate animal model with multiorgan pathology characteristic of the human form of cystic fibrosis has hampered our understanding of the pathobiology of the disease. We evaluated multiple organs of congenic C57BL/6J cystic fibrosis transmembrane regulator (Cftr)(-/-) and Cftr(+/+) mice maintained from weaning on a liquid diet then sacrificed between 1 and 24 months of age. The lungs of the Cftr(-/-) animals showed patchy alveolar overdistention, interstitial thickening, and fibrosis, with progression up to 6 months of age. The proximal and distal airway surface was encased with mucus-like material but lacked overt evidence of chronic bacterial infections or inflammation. All Cftr(-/-) animals showed progressive liver disease, with hepatosteatosis, focal cholangitis, inspissated secretions, and bile duct proliferation; after 1 year of age there was progression to focal biliary cirrhosis. The intercalated, intralobular and interlobular ducts and acinar lumina of the exocrine pancreas, the parotid and submaxillary glands of the Cftr(-/-) animals were dilated and filled with inspissated material, as well as mild inflammation and acinar cell drop out. Quantitative measurements of the pancreas showed significant acinar atrophy and increased acinar volume in comparison with age-matched Cftr(+/+) littermates. The ileal lumen and crypts were filled with adherent fibrillar material. After 3 months of age the vas deferens of the Cftr(-/-) animals could not be identified. None of the aforementioned pathological changes were observed in the Cftr(+/+) littermates fed the same liquid diet. We show, for the first time, that long-lived C578L/6J Cftr(-/-) mice develop manifestations of cystic fibrosis-like disease in all pathologically affected organs in the human form of cystic fibrosis.
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