Journal article
New animal models of cystic fibrosis: what are they teaching us?
Current opinion in pulmonary medicine, Vol.17(6), pp.478-483
11/2011
DOI: 10.1097/MCP.0b013e32834b14c9
PMCID: PMC3596000
PMID: 21857224
Abstract
Cystic fibrosis is the first human genetic disease to benefit from the directed engineering of three different species of animal models (mice, pigs, and ferrets). Recent studies on the cystic fibrosis pig and ferret models are providing new information about the pathophysiology of cystic fibrosis in various organ systems. Additionally, new conditional cystic fibrosis transmembrane conductance regulator (CFTR) knockout mice are teaching unexpected lessons about CFTR function in surprising cellular locations. Comparisons between these animal models and the human condition are key to dissecting the complexities of disease pathophysiology in cystic fibrosis.
Cystic fibrosis pigs and ferrets have provided new models to study the spontaneous development of disease in the lung and pancreas, two organs that are largely spared overt spontaneous disease in cystic fibrosis mice. New cystic fibrosis mouse models are now interrogating CFTR functions involved in growth and inflammation at an organ-based level using conditional knockout technology. Together, these models are providing new insights on the human condition.
Basic and clinical cystic fibrosis research will benefit greatly from the comparative pathophysiology of cystic fibrosis mice, pigs, and ferrets. Both similarities and differences between these three cystic fibrosis models will inform pathophysiologically important mechanisms of CFTR function in humans and aid in the development of both organ-specific and general therapies for cystic fibrosis.
Details
- Title: Subtitle
- New animal models of cystic fibrosis: what are they teaching us?
- Creators
- Nicholas W Keiser - Department of Anatomy and Cell Biology, University of Iowa Carver College of Medicine, Iowa City, Iowa 52242-1109, USAJohn F Engelhardt
- Resource Type
- Journal article
- Publication Details
- Current opinion in pulmonary medicine, Vol.17(6), pp.478-483
- DOI
- 10.1097/MCP.0b013e32834b14c9
- PMID
- 21857224
- PMCID
- PMC3596000
- NLM abbreviation
- Curr Opin Pulm Med
- ISSN
- 1070-5287
- eISSN
- 1531-6971
- Publisher
- United States
- Grant note
- R01 HL108902 / NHLBI NIH HHS RC1HL099516 / NHLBI NIH HHS P01 HL091842 / NHLBI NIH HHS DK047967 / NIDDK NIH HHS HL091842 / NHLBI NIH HHS R37 DK047967 / NIDDK NIH HHS R01 DK047967 / NIDDK NIH HHS RC1 HL099516 / NHLBI NIH HHS P30 DK054759 / NIDDK NIH HHS R24 DK091211 / NIDDK NIH HHS R24DK091211 / NIDDK NIH HHS
- Language
- English
- Date published
- 11/2011
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Anatomy and Cell Biology; Radiation Oncology; Internal Medicine
- Record Identifier
- 9984025364002771
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