2024
219 CFTR dysfunction shapes airway immune cell compositions contributing to lung pathogenesis in children with cystic fibrosis
Kizilirmak T, Yin H, Garrison A, Browne J, Bruscia E, Egan M, Britto C. 219 CFTR dysfunction shapes airway immune cell compositions contributing to lung pathogenesis in children with cystic fibrosis. Journal Of Cystic Fibrosis 2024, 23: s119. DOI: 10.1016/s1569-1993(24)01059-2.Peer-Reviewed Original Research
2011
Calcium-Modulated Chloride Pathways Contribute to Chloride Flux in Murine Cystic Fibrosis-Affected Macrophages
Shenoy A, Kopic S, Murek M, Caputo C, Geibel JP, Egan ME. Calcium-Modulated Chloride Pathways Contribute to Chloride Flux in Murine Cystic Fibrosis-Affected Macrophages. Pediatric Research 2011, 70: 447-452. PMID: 21796019, PMCID: PMC3189336, DOI: 10.1203/pdr.0b013e31822f2448.Peer-Reviewed Original ResearchConceptsCystic fibrosisWT macrophagesCF macrophagesExtracellular Ca2CAMP-activated chloride channelCystic fibrosis transmembrane conductance regulator (CFTR) proteinContribution of CFTRIon transport abnormalitiesResult of mutationsContribution of calciumRegulator proteinRobust inflammationChronic infectionMacrophage dysfunctionMacrophage functionIntracellular Ca2Transport abnormalitiesMacrophagesChloride channelsMethoxy-quinolinium bromideCFTRinh-172Fluorescent indicator dyesFibrosisPathwayCFTR
1998
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator
Schwiebert E, Morales M, Devidas S, Egan M, Guggino W. Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proceedings Of The National Academy Of Sciences Of The United States Of America 1998, 95: 2674-2679. PMID: 9482946, PMCID: PMC19458, DOI: 10.1073/pnas.95.5.2674.Peer-Reviewed Original ResearchMeSH Keywords4,4'-Diisothiocyanostilbene-2,2'-Disulfonic AcidAnimalsBase SequenceBronchiCells, CulturedChloride ChannelsChloridesCyclic AMPCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorDNA, ComplementaryEpithelial CellsFemaleHumansMembrane PotentialsModels, MolecularMolecular Sequence DataMutagenesis, Site-DirectedOligodeoxyribonucleotidesOocytesPatch-Clamp TechniquesPoint MutationProtein ConformationRecombinant ProteinsSequence DeletionTranscription, GeneticTransfectionXenopus laevisConceptsCl- channel functionConductance regulatorDomains of CFTRCystic fibrosis transmembrane conductance regulatorChloride channelsFibrosis transmembrane conductance regulatorFirst transmembrane domainC-terminal truncationsIndividual amino acid substitutionsTransmembrane conductance regulatorCl- channel poreCl- channelsAmino acid substitutionsRegulator domainTransmembrane domainTwo-electrode voltage-clamp recordingsRegulatory domainMutant CFTRAcid substitutionsRegulator functionHuman airway epithelial cellsCFTRXenopus oocytesRegulatorRelease of ATP
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
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP
Schwiebert E, Egan M, Hwang T, Fulmer S, Allen S, Cutting G, Guggino W. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 1995, 81: 1063-1073. PMID: 7541313, DOI: 10.1016/s0092-8674(05)80011-x.Peer-Reviewed Original ResearchConceptsUnknown regulatory mechanismCystic fibrosis transmembrane conductance regulator (CFTR) functionRegulatory mechanismsConductance regulatorCl- secretory pathwaySignaling mechanismShort-circuit current recordingsRegulator functionCFTR functionChloride channelsCellular mechanismsSingle-channel patch-clamp recordingsCFTRCl- channelsEpithelial cellsATPAutocrine mechanismCurrent recordingsORCCPathwayCF airwaysPatch-clamp recordingsCellsMechanismRegulatorDifferential 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
1994
Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes
Carroll T, Mclntosh I, Egan M, Zeitlin P, Cutting G, Guggino W. Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes. Cellular Physiology And Biochemistry 1994, 4: 10-18. DOI: 10.1159/000154705.Peer-Reviewed Original ResearchCystic fibrosis transmembrane conductance regulatorFibrosis transmembrane conductance regulatorWild-type CFTRXenopus oocytesAnti-CFTR antibodiesTransmembrane conductance regulatorCFTR Cl- channelTransmembrane regionMutant CFTRMutant formsMutation altersConductance regulatorCFTR mRNACl- currentReduced cAMPCF disease severityCl- channelsOpen channel probabilityWestern blottingCFTROocytesMutationsCl- conductanceChannel propertiesCAMP
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