File Download
  Links for fulltext
     (May Require Subscription)
Supplementary

Article: Lung disease in mice with cystic fibrosis

TitleLung disease in mice with cystic fibrosis
Authors
KeywordsChloride channels
Ion transport
Lung mechanics
Obstructive airway disease
Pulmonary fibrosis
Issue Date1997
PublisherAmerican Society for Clinical Investigation. The Journal's web site is located at http://www.jci.org
Citation
Journal Of Clinical Investigation, 1997, v. 100 n. 12, p. 3060-3069 How to Cite?
AbstractThe leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have bee compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite the presence of severe intestinal disease, to develop lung disease. Herein, we describe the phenotype of an inbred congenic strain of CFTR- knock-out mouse that develops spontaneous and progressive lung disease of early onset. The major features of the lung disease include failure of effective mucociliary transport, postbronchiolar over inflation of alveoli and parenchymal interstitial thickening, with evidence of fibrosis and inflammatory cell recruitment. We speculate that the basis for development of lung disease in the congenic CFTR-knockout mice is their observed lack of a non-CFTR chloride channel normally found in CFTR-knockout mice of mixed genetic background.
Persistent Identifierhttp://hdl.handle.net/10722/42307
ISSN
2015 Impact Factor: 12.575
2015 SCImago Journal Rankings: 8.764
PubMed Central ID
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorKent, Gen_HK
dc.contributor.authorIles, Ren_HK
dc.contributor.authorBear, CEen_HK
dc.contributor.authorHuan, LJen_HK
dc.contributor.authorGriesenbach, Uen_HK
dc.contributor.authorMcKerlie, Cen_HK
dc.contributor.authorFrndova, Hen_HK
dc.contributor.authorAckerley, Cen_HK
dc.contributor.authorGosselin, Den_HK
dc.contributor.authorRadzioch, Den_HK
dc.contributor.authorO'Brodovich, Hen_HK
dc.contributor.authorTsui, LCen_HK
dc.contributor.authorBuchwald, Men_HK
dc.contributor.authorTanswell, AKen_HK
dc.date.accessioned2007-01-08T02:34:11Z-
dc.date.available2007-01-08T02:34:11Z-
dc.date.issued1997en_HK
dc.identifier.citationJournal Of Clinical Investigation, 1997, v. 100 n. 12, p. 3060-3069en_HK
dc.identifier.issn0021-9738en_HK
dc.identifier.urihttp://hdl.handle.net/10722/42307-
dc.description.abstractThe leading cause of mortality and morbidity in humans with cystic fibrosis is lung disease. Advances in our understanding of the pathogenesis of the lung disease of cystic fibrosis, as well as development of innovative therapeutic interventions, have bee compromised by the lack of a natural animal model. The utility of the CFTR-knockout mouse in studying the pathogenesis of cystic fibrosis has been limited because of their failure, despite the presence of severe intestinal disease, to develop lung disease. Herein, we describe the phenotype of an inbred congenic strain of CFTR- knock-out mouse that develops spontaneous and progressive lung disease of early onset. The major features of the lung disease include failure of effective mucociliary transport, postbronchiolar over inflation of alveoli and parenchymal interstitial thickening, with evidence of fibrosis and inflammatory cell recruitment. We speculate that the basis for development of lung disease in the congenic CFTR-knockout mice is their observed lack of a non-CFTR chloride channel normally found in CFTR-knockout mice of mixed genetic background.en_HK
dc.format.extent968138 bytes-
dc.format.extent30208 bytes-
dc.format.mimetypeapplication/pdf-
dc.format.mimetypeapplication/msword-
dc.languageengen_HK
dc.publisherAmerican Society for Clinical Investigation. The Journal's web site is located at http://www.jci.orgen_HK
dc.relation.ispartofJournal of Clinical Investigationen_HK
dc.rightsCreative Commons: Attribution 3.0 Hong Kong License-
dc.subjectChloride channelsen_HK
dc.subjectIon transporten_HK
dc.subjectLung mechanicsen_HK
dc.subjectObstructive airway diseaseen_HK
dc.subjectPulmonary fibrosisen_HK
dc.subject.meshCystic fibrosis - pathology - physiopathologyen_HK
dc.subject.meshCystic fibrosis transmembrane conductance regulator - genetics - metabolismen_HK
dc.subject.meshDisease models, animalen_HK
dc.subject.meshElectrophysiologyen_HK
dc.subject.meshLung/microbiology - pathology - physiopathology - ultrastructureen_HK
dc.titleLung disease in mice with cystic fibrosisen_HK
dc.typeArticleen_HK
dc.identifier.openurlhttp://library.hku.hk:4550/resserv?sid=HKU:IR&issn=0021-9738&volume=100&issue=12&spage=3060&epage=3069&date=1997&atitle=Lung+disease+in+mice+with+cystic+fibrosisen_HK
dc.identifier.emailTsui, LC: tsuilc@hkucc.hku.hken_HK
dc.identifier.authorityTsui, LC=rp00058en_HK
dc.description.naturepublished_or_final_versionen_HK
dc.identifier.doi10.1172/JCI119861-
dc.identifier.pmid9399953-
dc.identifier.pmcidPMC508519-
dc.identifier.scopuseid_2-s2.0-0031438318en_HK
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0031438318&selection=ref&src=s&origin=recordpageen_HK
dc.identifier.volume100en_HK
dc.identifier.issue12en_HK
dc.identifier.spage3060en_HK
dc.identifier.epage3069en_HK
dc.identifier.isiWOS:000071149100019-
dc.publisher.placeUnited Statesen_HK
dc.identifier.scopusauthoridKent, G=34770664200en_HK
dc.identifier.scopusauthoridIles, R=24070881200en_HK
dc.identifier.scopusauthoridBear, CE=7006718679en_HK
dc.identifier.scopusauthoridHuan, LJ=7003602840en_HK
dc.identifier.scopusauthoridGriesenbach, U=6701826068en_HK
dc.identifier.scopusauthoridMcKerlie, C=35993902900en_HK
dc.identifier.scopusauthoridFrndova, H=6603616474en_HK
dc.identifier.scopusauthoridAckerley, C=7006092057en_HK
dc.identifier.scopusauthoridGosselin, D=7003373462en_HK
dc.identifier.scopusauthoridRadzioch, D=7007012582en_HK
dc.identifier.scopusauthoridO'Brodovich, H=7006287464en_HK
dc.identifier.scopusauthoridTsui, LC=7102754167en_HK
dc.identifier.scopusauthoridBuchwald, M=7006759922en_HK
dc.identifier.scopusauthoridTanswell, AK=35231349600en_HK

Export via OAI-PMH Interface in XML Formats


OR


Export to Other Non-XML Formats