2008
Rectal Potential Difference and the Functional Expression of CFTR in the Gastrointestinal Epithelia in Cystic Fibrosis Mouse Models
Weiner SA, Caputo C, Bruscia E, Ferreira EC, Price JE, Krause DS, Egan ME. Rectal Potential Difference and the Functional Expression of CFTR in the Gastrointestinal Epithelia in Cystic Fibrosis Mouse Models. Pediatric Research 2008, 63: 73-78. PMID: 18043508, DOI: 10.1203/pdr.0b013e31815b4bc6.Peer-Reviewed Original ResearchConceptsRectal potential differenceMouse modelCF mouse modelsCystic fibrosisFibrosis mouse modelDifferent mouse modelsCystic fibrosis mouse modelUssing chamber methodEffects of interventionsAutosomal recessive diseasePharmacologic interventionsRespiratory epitheliumElectrophysiologic phenotypeGastrointestinal epitheliumCF transmembrane conductance regulator (CFTR) geneRecessive diseaseVivo methodsVivo assaysVivo dataCFTR functionTransmembrane conductance regulator geneReliable assayEpitheliumInterventionCFTR expression
2004
Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects
Egan ME, Pearson M, Weiner SA, Rajendran V, Rubin D, Glöckner-Pagel J, Canny S, Du K, Lukacs GL, Caplan MJ. Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects. Science 2004, 304: 600-602. PMID: 15105504, DOI: 10.1126/science.1093941.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCalciumCalnexinCell LineCell MembraneCricetinaeCurcuminCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorElectrolytesEndoplasmic ReticulumGene TargetingGlycosylationHumansIntestinal MucosaIntestinal ObstructionIsoproterenolMembrane PotentialsMiceMice, KnockoutMutationNasal MucosaPolyethylene GlycolsProtein FoldingRectumTransfectionConceptsCystic fibrosis transmembrane conductance regulatorCFTR proteinDeltaF508 cystic fibrosis transmembrane conductance regulatorDeltaF508 CFTR proteinFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorBaby hamster kidney cellsPlasma membraneComplete knockoutConductance regulatorHamster kidney cellsEndoplasmic reticulumCystic fibrosis defectCFTR geneKidney cellsCFTR miceGenesProteinMutationsCommon mutationsHomozygous expressionCurcumin treatmentFunctional appearanceWeight basisRegulator
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[49] Assays of dynamics, mechanisms, and regulation of ATP transport and release: Implications for study of ABC transporter function
Schwiebert E, Egan M, Guggino W. [49] Assays of dynamics, mechanisms, and regulation of ATP transport and release: Implications for study of ABC transporter function. Methods In Enzymology 1998, 292: 664-675. PMID: 9711590, DOI: 10.1016/s0076-6879(98)92051-1.Peer-Reviewed Original ResearchMeSH Keywords3T3 CellsAdenosine TriphosphateAnimalsATP-Binding Cassette TransportersCells, CulturedColforsinCystic Fibrosis Transmembrane Conductance RegulatorElectrophysiologyEpithelial CellsHumansIonomycinLuminescent MeasurementsMembrane PotentialsMiceModels, BiologicalOocytesOsmolar ConcentrationPatch-Clamp TechniquesSignal TransductionTritiumConceptsCystic fibrosis transmembrane conductance regulatorABC transportersATP-binding cassette (ABC) transportersSulfonylurea receptorFibrosis transmembrane conductance regulatorTransport of ATPABC transporter functionTransmembrane conductance regulatorImportance of ATPRegulatory machineryPancreatic β-cellsATP transportCassette transportersConductance regulatorTransporter functionTransporter moleculesBiological significanceATP sensorATPAgonist functionTransportersRelease of ATPΒ-cellsPowerful approachRegulator
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 mutationA functional CFTR-NBF1 is required for ROMK2-CFTR interaction
McNicholas C, Nason M, Guggino W, Schwiebert E, Hebert S, Giebisch G, Egan M. A functional CFTR-NBF1 is required for ROMK2-CFTR interaction. American Journal Of Physiology 1997, 273: f843-f848. PMID: 9374850, DOI: 10.1152/ajprenal.1997.273.5.f843.Peer-Reviewed Original ResearchAmino Acid SubstitutionAnimalsBase SequenceBinding SitesCystic Fibrosis Transmembrane Conductance RegulatorFemaleGlyburideMembrane PotentialsModels, MolecularMutagenesis, Site-DirectedOligodeoxyribonucleotidesOocytesPatch-Clamp TechniquesPotassium ChannelsPotassium Channels, Inwardly RectifyingProtein Structure, SecondaryRecombinant ProteinsXenopus laevis
1996
Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator.
McNicholas C, Guggino W, Schwiebert E, Hebert S, Giebisch G, Egan M. Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator. Proceedings Of The National Academy Of Sciences Of The United States Of America 1996, 93: 8083-8088. PMID: 8755607, PMCID: PMC38879, DOI: 10.1073/pnas.93.15.8083.Peer-Reviewed Original ResearchMeSH KeywordsAdenosine TriphosphateAnimalsChloride ChannelsCyclic AMP-Dependent Protein KinasesCystic Fibrosis Transmembrane Conductance RegulatorFemaleGlyburideKidneyMembrane PotentialsOocytesPatch-Clamp TechniquesPhosphorylationPotassium ChannelsPotassium Channels, Inwardly RectifyingSodium ChannelsXenopus laevis
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 recordingsCellsMechanismRegulator
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