Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased mucociliary clearance, bacterial colonisation, and chronic neutrophil-dominated inflammation. Much knowledge surr...

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Main Authors: Gillian M. Lavelle, Michelle M. White, Niall Browne, Noel G. McElvaney, Emer P. Reeves
Format: Article
Language:English
Published: Hindawi Limited 2016-01-01
Series:BioMed Research International
Online Access:http://dx.doi.org/10.1155/2016/5258727
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spelling doaj-4569b5916d324ecbabda3efce03ab4842020-11-24T23:50:18ZengHindawi LimitedBioMed Research International2314-61332314-61412016-01-01201610.1155/2016/52587275258727Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and DivergencesGillian M. Lavelle0Michelle M. White1Niall Browne2Noel G. McElvaney3Emer P. Reeves4Respiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, IrelandRespiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, IrelandRespiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, IrelandRespiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, IrelandRespiratory Research Division, Department of Medicine, Royal College of Surgeons in Ireland, Beaumont Hospital, Dublin 9, IrelandCystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased mucociliary clearance, bacterial colonisation, and chronic neutrophil-dominated inflammation. Much knowledge surrounding the pathophysiology of the disease has been gained through the generation of animal models, despite inherent limitations in each. The failure of certain mouse models to recapitulate the phenotypic manifestations of human disease has initiated the generation of larger animals in which to study CF, including the pig and the ferret. This review will summarise the basic phenotypes of three animal models and describe the contributions of such animal studies to our current understanding of CF.http://dx.doi.org/10.1155/2016/5258727
collection DOAJ
language English
format Article
sources DOAJ
author Gillian M. Lavelle
Michelle M. White
Niall Browne
Noel G. McElvaney
Emer P. Reeves
spellingShingle Gillian M. Lavelle
Michelle M. White
Niall Browne
Noel G. McElvaney
Emer P. Reeves
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
BioMed Research International
author_facet Gillian M. Lavelle
Michelle M. White
Niall Browne
Noel G. McElvaney
Emer P. Reeves
author_sort Gillian M. Lavelle
title Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
title_short Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
title_full Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
title_fullStr Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
title_full_unstemmed Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences
title_sort animal models of cystic fibrosis pathology: phenotypic parallels and divergences
publisher Hindawi Limited
series BioMed Research International
issn 2314-6133
2314-6141
publishDate 2016-01-01
description Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The resultant characteristic ion transport defect results in decreased mucociliary clearance, bacterial colonisation, and chronic neutrophil-dominated inflammation. Much knowledge surrounding the pathophysiology of the disease has been gained through the generation of animal models, despite inherent limitations in each. The failure of certain mouse models to recapitulate the phenotypic manifestations of human disease has initiated the generation of larger animals in which to study CF, including the pig and the ferret. This review will summarise the basic phenotypes of three animal models and describe the contributions of such animal studies to our current understanding of CF.
url http://dx.doi.org/10.1155/2016/5258727
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AT michellemwhite animalmodelsofcysticfibrosispathologyphenotypicparallelsanddivergences
AT niallbrowne animalmodelsofcysticfibrosispathologyphenotypicparallelsanddivergences
AT noelgmcelvaney animalmodelsofcysticfibrosispathologyphenotypicparallelsanddivergences
AT emerpreeves animalmodelsofcysticfibrosispathologyphenotypicparallelsanddivergences
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