ArticleThe EMBO journal1995
A mouse model for the cystic fibrosis delta F508 mutation.
Article in The EMBO journal, 1995. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 109 papers, 1 of them a synthesis that pooled 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.
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.
Who cites it
109 citing papers in PubMed, 1 synthesis or guideline pooled it, 237 citations in OpenAlex.
- A systematic review of animal and human data comparing the nasal potential difference test between cystic fibrosis and control.Scientific reports · 2024Pooled it
- A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR.Cell death and differentiation · 2016Trial
- Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation.Autophagy · 2014Trial
- Male reproductive phenotype in cystic fibrosis: comparison of existing animal models.Lab animal · 2026Review
- Restoring chloride efflux in cystic fibrosis with TMEM16a antisense oligonucleotides.Molecular therapy : the journal of the American Society of Gene Therapy · 2025Article
- Condition-dependent effects of Elexacaftor/Tezacaftor/Ivacaftor (Trikafta) onMicrobiology spectrum · 2025Article
- Endometrium-derived organoids from cystic fibrosis patients and mice as new models to study disease-associated endometrial pathobiology.Cellular and molecular life sciences : CMLS · 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
- The anion exchanger slc26a3 regulates colonic mucus expansion during steady state and in response to prostaglandin EPflugers Archiv : European journal of physiology · 2024Article
- FGF receptors mediate cellular senescence in the cystic fibrosis airway epithelium.JCI insight · 2024Article
- Cystic Fibrosis Mice Are Highly Susceptible to Repeated AcuteBioMed research international · 2024Article
- Dual species sphingosine-1-phosphate lyase inhibitors to combine antifungal and anti-inflammatory activities in cystic fibrosis: a feasibility study.Scientific reports · 2023Article
- Bridging of host-microbiota tryptophan partitioning by the serotonin pathway in fungal pneumonia.Nature communications · 2023Article
- Changes in Essential Fatty Acids and Ileal Genes Associated with Metabolizing Enzymes and Fatty Acid Transporters in Rodent Models of Cystic Fibrosis.International journal of molecular sciences · 2023Article
- Coxsackievirus B infections are common in Cystic Fibrosis and experimental evidence supports protection by vaccination.iScience · 2022Article
- Clearance of small intestinal crypts involves goblet cell mucus secretion by intracellular granule rupture and enterocyte ion transport.Science signaling · 2022Article
- Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.Scientific reports · 2022Article
- Anakinra restores cellular proteostasis by coupling mitochondrial redox balance to autophagy.The Journal of clinical investigation · 2022Article
- Methodological tools to study species of the genus Burkholderia.Applied microbiology and biotechnology · 2021Review
- Role for animal models in understanding essential fatty acid deficiency in cystic fibrosis.Cellular and molecular life sciences : CMLS · 2021Review
49 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Most cystic fibrosis (CF) patients produce a mutant form (delta F508) of the cystic fibrosis transmembrane conductance regulator (CFTR), which is not properly processed in normal cells but is active as a chloride channel in several experimental systems. We used a double homologous recombination ('Hit and Run') procedure to generate a mouse model for the delta F508 mutation. Targeted embryonic stem (ES) cells (Hit clones) were found; of these either 80 or 20% of the clones had lost the delta F508 mutation, depending on the distance between the linearization site in the targeting construct and the delta F508 mutation. Correctly targeted clones underwent a second selection step resulting in ES cell clones (Run clones) heterozygous for the delta F508 mutation with an efficiency of 2-7%. Chimeric mice were generated and offspring homozygous for the delta F508 mutation showed electrophysiological abnormalities in nasal epithelium, gallbladder and in the intestine, and histological abnormalities in the intestine, typical of CF. Our data suggest that the delta F508 mice have residual delta F508 CFTR activity which would explain the mild pathology of the delta F508 mice. The delta F508 mouse may provide a useful model for the study of the processing defect of delta F508 CFTR and for the development of novel therapeutic approaches based on circumvention of the processing block.
Indexed as
Identifiers
What OpenQuestion holds
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.