ArticleThe Journal of clinical investigation1995
A mouse model for the delta F508 allele of cystic fibrosis.
Article in The Journal of clinical investigation, 1995. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 141 papers.
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Who cites it
141 citing papers in PubMed.
- Male reproductive phenotype in cystic fibrosis: comparison of existing animal models.Lab animal · 2026Review
- Advancing Gene Therapy for Pancreatitis: From Genetic Insights to Clinical Translation.Research (Washington, D.C.) · 2026Review
- Systemic in utero gene editing as a treatment for cystic fibrosis.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Increased NFAT and NFκB signalling contribute to the hyperinflammatory phenotype in response to Aspergillus fumigatus in a mouse model of cystic fibrosis.PLoS pathogens · 2025Article
- Think Beyond the Room: Measuring Relative Humidity in the Home Cage and Its Impact on Reproduction in Laboratory Mice,Animals : an open access journal from MDPI · 2024Article
- Autophagy in Disease Onset and Progression.Aging and disease · 2024Review
- CFTR dysfunction in smooth muscle drives TGFβ dependent airway hyperreactivity.Respiratory research · 2023Article
- CFTR regulates brown adipocyte thermogenesis via the cAMP/PKA signaling pathway.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2023Article
- The human ATP-binding cassette (ABC) transporter superfamily.Human mutation · 2022Review
- Bicarbonate transport of airway surface epithelia in luminally perfused mice bronchioles.The journal of physiological sciences : JPS · 2022Article
- Bone Marrow Transplantation Rescues Monocyte Recruitment Defect and Improves Cystic Fibrosis in Mice.Journal of immunology (Baltimore, Md. : 1950) · 2022Article
- Investigating the Implications ofFrontiers in pharmacology · 2022Article
- CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages.Frontiers in cellular and infection microbiology · 2022Article
- Methodological tools to study species of the genus Burkholderia.Applied microbiology and biotechnology · 2021Review
- Serum inflammatory profiles in cystic fibrosis mice with and without Bordetella pseudohinzii infection.Scientific reports · 2021Article
- Quorum sensing inhibitory effect of bergamot oil and aspidosperma extract against Chromobacterium violaceum and Pseudomonas aeruginosa.Archives of microbiology · 2021Article
- Therapeutic Uses of Bacterial Subunit Toxins.Toxins · 2021Review
- Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation.Molecular therapy : the journal of the American Society of Gene Therapy · 2021Article
- Gene Editing in Rabbits: Unique Opportunities for Translational Biomedical Research.Frontiers in genetics · 2021Review
- On the Corner of Models and Cure: Gene Editing in Cystic Fibrosis.Frontiers in pharmacology · 2021Review
81 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
9 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The most common cause of cystic fibrosis is a mutation that deletes phenylalanine 508 in cystic fibrosis transmembrane conductance regulator (CFTR). The delta F508 protein is misprocessed and degraded rather than traveling to the apical membrane. We used a novel strategy to introduce the delta F508 mutation into the mouse CFTR gene. Affected epithelia from homozygous delta F508 mice lacked CFTR in the apical membrane and were Cl-impermeable. These abnormalities are the same as those observed in patients with delta F508 and suggest that these mice have the same cellular defect. 40% of homozygous delta F508 animals survived into adulthood and displayed several abnormalities found in human disease and in CFTR null mice. These animals should provide an excellent model to investigate pathogenesis and to examine therapies directed at correcting the delta F508 defect.
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