ArticleProceedings of the National Academy of Sciences of the United States of America2013
CFTR-deficient pigs display peripheral nervous system defects at birth.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 52 citations in OpenAlex.
- Structural and functional impact of cystic fibrosis on endocrine pancreatic innervation.Clinical science (London, England : 1979) · 2026Article
- Review
- Considerations for early life genetic therapies in cystic fibrosis.American journal of physiology. Lung cellular and molecular physiology · 2026Review
- Whole genome sequencing identifies genetic variants associated with neurogenic inflammation in rosacea.Nature communications · 2023Article
- Genome Editing for Cystic Fibrosis.Cells · 2023Review
- Testing spatial working memory in pigs using an automated T-maze.Oxford open neuroscience · 2023Article
- Identification of cholinergic cells with chemosensory traits in the porcine uterus.Cell and tissue research · 2022Article
- Impact of Physical Activity on Cognitive Functions: A New Field for Research and Management of Cystic Fibrosis.Diagnostics (Basel, Switzerland) · 2020Review
- CFTR promotes malignant glioma development via up-regulation of Akt/Bcl2-mediated anti-apoptosis pathway.Journal of cellular and molecular medicine · 2020Article
- Long-term culturing of porcine nodose ganglia.Journal of neuroscience methods · 2020Article
- Chloride - The Underrated Ion in Nociceptors.Frontiers in neuroscience · 2020Review
- Cell-Selective Regulation of CFTR Gene Expression: Relevance to Gene Editing Therapeutics.Genes · 2019Review
- Sex-specific airway hyperreactivity and sex-specific transcriptome remodeling in neonatal piglets challenged with intra-airway acid.American journal of physiology. Lung cellular and molecular physiology · 2019Article
- CNS imaging studies in cystic fibrosis patients presenting with sudden neurological events.BMJ open respiratory research · 2019Article
- Neuropeptides in asthma, chronic obstructive pulmonary disease and cystic fibrosis.Respiratory research · 2018Review
- Development of a polarized pancreatic ductular cell epithelium for physiological studies.Journal of applied physiology (Bethesda, Md. : 1985) · 2018Article
- Article
- Genetically engineered pigs as models for human disease.Disease models & mechanisms · 2018Review
- A role for the cystic fibrosis transmembrane conductance regulator in the nitric oxide-dependent release of ClJournal of neurophysiology · 2017Article
- Simple and reproducible approaches for the collection of select porcine ganglia.Journal of neuroscience methods · 2017Article
Corrections and comments
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Authors and funding
12 authors at 6 institutions in 3 countries.
Funding
Abstract
Peripheral nervous system abnormalities, including neuropathy, have been reported in people with cystic fibrosis. These abnormalities have largely been attributed to secondary manifestations of the disease. We tested the hypothesis that disruption of the cystic fibrosis transmembrane conductance regulator (CFTR) gene directly influences nervous system function by studying newborn CFTR(-/-) pigs. We discovered CFTR expression and activity in Schwann cells, and loss of CFTR caused ultrastructural myelin sheath abnormalities similar to those in known neuropathies. Consistent with neuropathic changes, we found increased transcripts for myelin protein zero, a gene that, when mutated, can cause axonal and/or demyelinating neuropathy. In addition, axon density was reduced and conduction velocities of the trigeminal and sciatic nerves were decreased. Moreover, in vivo auditory brainstem evoked potentials revealed delayed conduction of the vestibulocochlear nerve. Our data suggest that loss of CFTR directly alters Schwann cell function and that some nervous system defects in people with cystic fibrosis are likely primary.
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