1997
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.
Rubenstein R, Egan M, Zeitlin P. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. Journal Of Clinical Investigation 1997, 100: 2457-2465. PMID: 9366560, PMCID: PMC508446, DOI: 10.1172/jci119788.Peer-Reviewed Original ResearchConceptsDelta F508-CFTRIB3-1 cellsCystic fibrosis epithelial cellsFunctional chloride channelChloride channelsHigh molecular mass formsModification of oligosaccharidesCF epithelial cellsEpithelial cellsMolecular mass formsTranscriptional regulatorsPlasma membraneGolgi apparatusEndoplasmic reticulumCystic fibrosis transmembrane conductance regulator (CFTR) mutationsCF phenotypeIB3-1Cell surfaceDelta F508 mutationWhole cellsMass formNasal polyp epitheliumRegulator mutationsCFTRF508 mutation
1995
Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells
Egan M, Schwiebert E, Guggino W. Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells. American Journal Of Physiology 1995, 268: c243-c251. PMID: 7530908, DOI: 10.1152/ajpcell.1995.268.1.c243.Peer-Reviewed Original ResearchConceptsCl- channel activityAirway epithelial cellsEpithelial cellsCFTR Cl- channel activityProtein kinase ANonepithelial cell typesMutant proteinsKinase ACF airway epithelial cellsIon channel expressionDifferential expressionIncubation temperatureCell typesCF airway epitheliaChannel expressionRegulator mutationsCFTRMultiple alterationsMutationsCells