ArticleJournal of molecular medicine (Berlin, Germany)2012
Sequential targeting of CFTR by BAC vectors generates a novel pig model of cystic fibrosis.
Article in Journal of molecular medicine (Berlin, Germany), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
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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.
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Who cites it
50 citing papers in PubMed, 97 citations in OpenAlex.
- Male reproductive phenotype in cystic fibrosis: comparison of existing animal models.Lab animal · 2026Review
- Activating a patient-relevant pathogenic variant of a genetically defined heart disease in a humanized pig model.Lab animal · 2026Article
- From farm to lab: gene-edited sheep transforming bone research.JBMR plus · 2026Review
- Considerations for early life genetic therapies in cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2026Review
- Cystic fibrosis alters the structure of the olfactory epithelium and the expression of olfactory receptors affecting odor perception.Science advances · 2025Article
- Mapping the oxidative landscape in cystic fibrosis: methodological frontiers and application.Frontiers in pharmacology · 2025Review
- Pathogenic Relationships in Cystic Fibrosis and Renal Diseases: CFTR, SLC26A9 and Anoctamins.International journal of molecular sciences · 2023Review
- Generation of sheep with defined FecBBMC genomics · 2023Article
- Proteome of airway surface liquid and mucus in newborn wildtype and cystic fibrosis piglets.Respiratory research · 2023Article
- Computational, Ex Vivo, and Tissue Engineering Techniques for Modeling Large Airways.Advances in experimental medicine and biology · 2023Article
- Early disruption of photoreceptor cell architecture and loss of vision in a humanized pig model of usher syndromes.EMBO molecular medicine · 2022Article
- Expression of SLC26A9 in Airways and Its Potential Role in Asthma.International journal of molecular sciences · 2022Article
- Use of Agriculturally Important Animals as Models in Biomedical Research.Advances in experimental medicine and biology · 2022Review
- In vitro genome editing activity of Cas9 in somatic cells after random and transposon-based genomic Cas9 integration.PloS one · 2022Article
- Use of Translational, Genetically Modified Porcine Models to Ultimately Improve Intestinal Disease Treatment.Frontiers in veterinary science · 2022Review
- Airway Administration of Flagellin Regulates the Inflammatory Response toAmerican journal of respiratory cell and molecular biology · 2021Article
- New generation ENaC inhibitors detach cystic fibrosis airway mucus bundles via sodium/hydrogen exchanger inhibition.European journal of pharmacology · 2021Article
- CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells.Frontiers in physiology · 2021Article
- Transport properties in CFTR-/- knockout piglets suggest normal airway surface liquid pH and enhanced amiloride-sensitive NaPflugers Archiv : European journal of physiology · 2020Article
- Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
18 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Cystic fibrosis (CF) is the most common lethal inherited disease in Caucasians and is caused by mutations in the CFTR gene. The disease is incurable and medical treatment is limited to the amelioration of symptoms or secondary complications. A comprehensive understanding of the disease mechanisms and the development of novel treatment options require appropriate animal models. Existing CF mouse models fail to reflect important aspects of human CF. We thus generated a CF pig model by inactivating the CFTR gene in primary porcine cells by sequential targeting using modified bacterial artificial chromosome vectors. These cells were then used to generate homozygous CFTR mutant piglets by somatic cell nuclear transfer. The homozygous CFTR mutants lack CFTR protein expression and display severe malformations in the intestine, respiratory tract, pancreas, liver, gallbladder, and male reproductive tract. These phenotypic abnormalities closely resemble both the human CF pathology as well as alterations observed in a recently published CF pig model which was generated by a different gene targeting strategy. Our new CF pig model underlines the value of the CFTR-deficient pig for gaining new insight into the disease mechanisms of CF and for the development and evaluation of new therapeutic strategies. This model will furthermore increase the availability of CF pigs to the scientific community.
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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.