Airway Surface Liquid Volume Regulation Determines Different Airway Phenotypes in Liddle Compared with βENaC-overexpressing Mice

Studies in cystic fibrosis patients and mice overexpressing the epithelial Na(+) channel β-subunit (βENaC-Tg) suggest that raised airway Na(+) transport and airway surface liquid (ASL) depletion are central to the pathogenesis of cystic fibrosis lung disease. However, patients or mice with Liddle ga...

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Autors principals: Mall, Marcus A., Button, Brian, Johannesson, Bjarki, Zhou, Zhe, Livraghi, Alessandra, Caldwell, Ray A., Schubert, Susanne C., Schultz, Carsten, O'Neal, Wanda K., Pradervand, Sylvain, Hummler, Edith, Rossier, Bernard C., Grubb, Barbara R., Boucher, Richard C.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2010
Matèries:
Molecular Bases of Disease
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2930694/
https://ncbi.nlm.nih.gov/pubmed/20566636
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.151803
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