Beta-oestradiol rescues DeltaF508CFTR functional expression in human cystic fibrosis airway CFBE41o- cells through the up-regulation of NHERF1

CF (cystic fibrosis) is a disease caused by mutations within the CFTR (CF transmembrane conductance regulator) gene. The most common mutation, DeltaF508 (deletion of Phe-508), results in a protein that is defective in folding and trafficking to the cell surface but is functional if properly localize...

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Veröffentlicht in:	Biology of the cell 2008-07, Vol.100 (7), p.399
Hauptverfasser:	Fanelli, Teresa, Cardone, Rosa Angela, Favia, Maria, Guerra, Lorenzo, Zaccolo, Manuela, Monterisi, Stefania, De Santis, Teresa, Riccardi, Stefania Maria, Reshkin, Stephan Joel, Casavola, Valeria
Format:	Artikel
Sprache:	eng
Schlagworte:	Biological Transport Cells, Cultured Chlorides - metabolism Cyclic AMP-Dependent Protein Kinases - metabolism Cystic Fibrosis - genetics Cystic Fibrosis - metabolism Cystic Fibrosis Transmembrane Conductance Regulator - analysis Cystic Fibrosis Transmembrane Conductance Regulator - genetics Cystic Fibrosis Transmembrane Conductance Regulator - metabolism Estradiol - pharmacology Gene Expression - drug effects Humans Mutation Phosphoproteins - genetics Phosphoproteins - metabolism Respiratory Mucosa - drug effects Respiratory Mucosa - metabolism RNA, Small Interfering - genetics Sodium-Hydrogen Exchangers - genetics Sodium-Hydrogen Exchangers - metabolism Up-Regulation
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