ArticleTransactions of the American Clinical and Climatological Association2009
Development of a porcine model of cystic fibrosis.
Article in Transactions of the American Clinical and Climatological Association, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
What it found
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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.
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Who cites it
41 citing papers in PubMed, 79 citations in OpenAlex.
- Intranasal micro-optical coherence tomography imaging for cystic fibrosis studies.Science translational medicine · 2019Trial
- Farm animal models for the study of the reproductive features of polyendocrine metabolic ovarian syndrome.Frontiers in endocrinology · 2026Review
- Mapping the oxidative landscape in cystic fibrosis: methodological frontiers and application.Frontiers in pharmacology · 2025Review
- Chronic hyperglycemia aggravates lung function in aAmerican journal of physiology. Lung cellular and molecular physiology · 2024Article
- Pig Models in Retinal Research and Retinal Disease.Cold Spring Harbor perspectives in medicine · 2024Review
- Magnetic resonance brain volumetry biomarkers of CLN2 Batten disease identified with miniswine model.Scientific reports · 2023Article
- ASK1 is a novel molecular target for preventing aminoglycoside-induced hair cell death.Journal of molecular medicine (Berlin, Germany) · 2022Article
- Future of biomedical, agricultural, and biological systems research using domesticated animals.Biology of reproduction · 2022Article
- Biofilm aggregates and the host airway-microbial interface.Frontiers in cellular and infection microbiology · 2022Review
- A Novel AAV-mediated Gene Delivery System Corrects CFTR Function in Pigs.American journal of respiratory cell and molecular biology · 2019Article
- Therapeutic landscape for Batten disease: current treatments and future prospects.Nature reviews. Neurology · 2019Review
- Article
- Dynamic Changes of DNA Methylation and Transcriptome Expression in Porcine Ovaries during Aging.BioMed research international · 2019Article
- Silencing of retrotransposon-derived imprinted gene RTL1 is the main cause for postimplantational failures in mammalian cloning.Proceedings of the National Academy of Sciences of the United States of America · 2018Article
- Widespread airway distribution and short-term phenotypic correction of cystic fibrosis pigs following aerosol delivery of piggyBac/adenovirus.Nucleic acids research · 2018Article
- Comprehensive transcriptional landscape of porcine cardiac and skeletal muscles reveals differences of aging.Oncotarget · 2018Article
- Animal Models in the Pathophysiology of Cystic Fibrosis.Frontiers in pharmacology · 2018Review
- Effects of coconut oil on glycemia, inflammation, and urogenital microbial parameters in female Ossabaw mini-pigs.PloS one · 2017Article
- Lentiviral-mediated phenotypic correction of cystic fibrosis pigs.JCI insight · 2016Article
- Genome editing revolutionize the creation of genetically modified pigs for modeling human diseases.Human genetics · 2016Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
5 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic Fibrosis (CF) is a common autosomal recessive disease that affects multiple organs. The lack of an animal model with manifestations like those typically found in humans has slowed understanding of its pathogenesis. Therefore, because of the similarities between human and swine anatomy, biochemistry, physiology, size, and genetics, we chose to develop a porcine model of CF. We used homologous recombination in primary cultures of porcine fibroblasts to disrupt the CFTR gene and then used those cells as nuclear donors for somatic cell nuclear transfer. After crossing heterozygous pigs, we produced CFTR-/- pigs. The newborn CFTR null piglets manifested meconium ileus, pancreatic destruction, early focal biliary cirrhosis, and gall bladder abnormalities that were very similar to those observed in humans with CF. At birth, there were no abnormalities in the airway epithelium or submucosal glands and no evidence of inflammation, consistent with findings in the newborn human. We hope that this porcine model will help elucidate the pathogenesis of CF and thereby lead to the development of new mechanism-based therapies.
Indexed as
Identifiers
19768173PMC2744522W1547732194What 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.