2006
ΔF508 Mutation Results in Impaired Gastric Acid Secretion*
Sidani SM, Kirchhoff P, Socrates T, Stelter L, Ferreira E, Caputo C, Roberts KE, Bell RL, Egan ME, Geibel JP. ΔF508 Mutation Results in Impaired Gastric Acid Secretion*. Journal Of Biological Chemistry 2006, 282: 6068-6074. PMID: 17178714, DOI: 10.1074/jbc.m608427200.Peer-Reviewed Original ResearchConceptsCystic fibrosis transmembrane conductance regulatorATP-binding cassette (ABC) transportersFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorMouse gastric glandsParietal cellsMultifunctional proteinCFTR proteinRegulatory proteinsTransport proteinsCassette transportersConductance regulatorRegulatory roleApical poleSecretagogue-induced acid secretionGland lumenGastric glandsSulfonylurea receptorProteinImpaired gastric acid secretionK-ATPaseCl(-) secretionImmunofluorescent localizationCl- channelsATP-sensitive potassium channels
1999
CFTR Is a Conductance Regulator as well as a Chloride Channel
SCHWIEBERT E, BENOS D, EGAN M, STUTTS M, GUGGINO W. CFTR Is a Conductance Regulator as well as a Chloride Channel. Physiological Reviews 1999, 79: s145-s166. PMID: 9922379, DOI: 10.1152/physrev.1999.79.1.s145.Peer-Reviewed Original ResearchConceptsCystic fibrosis transmembrane conductance regulatorConductance regulatorABC transportersCassette transporter gene familyCFTR Cl- channel functionTransporter gene familyFamily of transportersChloride channelsFibrosis transmembrane conductance regulatorCl- channel functionABC transporter familyTransmembrane conductance regulatorIon channel proteinsCystic fibrosis epitheliaGene familyCellular functionsCellular proteinsTransporter familyChannel proteinsCF geneAmino acidsIon channelsRegulatorTransportersCl- channels
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
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
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