2002
Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells
Egan ME, Glöckner-Pagel J, Ambrose C, Cahill PA, Pappoe L, Balamuth N, Cho E, Canny S, Wagner CA, Geibel J, Caplan MJ. Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells. Nature Medicine 2002, 8: 485-492. PMID: 11984593, DOI: 10.1038/nm0502-485.Peer-Reviewed Original ResearchConceptsEndoplasmic reticulumCalcium pump inhibitorΔF508-CFTR proteinCystic fibrosis epithelial cellsCystic fibrosis transmembrane conductance regulator (CFTR) proteinCystic fibrosis cell lineFunctional surface expressionSurface expressionChaperone activityChaperone proteinsRegulator proteinPlasma membraneCystic fibrosis defectCell surfaceProteinCell linesPotential targetOptimal activityInhibitor thapsigarginEpithelial cellsExpressionCommon mutationsInhibitorsMouse modelReticulum
1992
Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTR
Egan M, Flotte T, Afione S, Solow R, Zeitlin P, Carter B, Guggino W. Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTR. Nature 1992, 358: 581-584. PMID: 1380129, DOI: 10.1038/358581a0.Peer-Reviewed Original ResearchConceptsCyclic AMP-dependent proteinCF geneCF bronchial epithelial cellsLethal genetic diseaseProtein kinaseDefective acidificationBronchial epithelial cellsGenetic diseasesProteinDefective regulationLarge conductanceEpithelial cellsGenesCFTRConductance pathwayCystic fibrosisLinear current-voltage relationshipVirus vectorsRegulationKinaseConductancePathwayExpressionAcidificationCells