ArticleProceedings of the National Academy of Sciences of the United States of America1995
Molecular analysis of the ovine cystic fibrosis transmembrane conductance regulator gene.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1995. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers.
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Who cites it
11 citing papers in PubMed.
- Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.Laboratory investigation; a journal of technical methods and pathology · 2018Article
- Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.The Journal of physiology · 2015Article
- Article
- Efficient gene delivery to pig airway epithelia and submucosal glands using helper-dependent adenoviral vectors.Molecular therapy. Nucleic acids · 2013Article
- Alternative splicing of the ovine CFTR gene.Mammalian genome : official journal of the International Mammalian Genome Society · 2003Article
- Atypical 5' splice sites cause CFTR exon 9 to be vulnerable to skipping.American journal of human genetics · 2002Article
- The ovine cathelicidin SMAP29 kills ovine respiratory pathogens in vitro and in an ovine model of pulmonary infection.Antimicrobial agents and chemotherapy · 2001Article
- In vivo analysis of DNase I hypersensitive sites in the human CFTR gene.Molecular medicine (Cambridge, Mass.) · 1999Article
- Genomic DNA sequence of Rhesus (M. mulatta) cystic fibrosis (CFTR) gene.Mammalian genome : official journal of the International Mammalian Genome Society · 1998Article
- Comparative genome organization of vertebrates. The First International Workshop on Comparative Genome Organization.Mammalian genome : official journal of the International Mammalian Genome Society · 1996Article
- An ovine CFTR variant as a putative cystic fibrosis causing mutation.Journal of medical genetics · 1996Article
Corrections and comments
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Authors and funding
4 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
There is a need for a large-animal model to investigate the etiology and biology of cystic fibrosis (CF) lung disease and to study potential therapies. The development and electrophysiology of the sheep airway have been shown to exhibit close functional parallels with the human airway, particularly with respect to the respiratory epithelium. We have cloned and sequenced the ovine cystic fibrosis transmembrane conductance regulator (CFTR) cDNA. It shows a high degree of conservation at the DNA coding and predicted polypeptide levels with human CFTR: at the nucleic acid level there is a 90% conservation (compared with 80% between human and mouse CFTR cDNA); at the polypeptide level, the degree of similarity is 95% (compared with 88% between human and mouse). Northern blot analysis and reverse transcription-PCR have shown that the patterns of expression of the ovine CFTR gene are very similar to those seen in humans. Further, the developmental expression of CFTR in the sheep is equivalent to that observed in humans. Thus, overall a CF sheep should show lung pathology similar to that of humans with CF.
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