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
2000
Identification of the Cystic Fibrosis Transmembrane Conductance Regulator Domains That Are Important for Interactions with ROMK2*
Cahill P, Nason M, Ambrose C, Yao T, Thomas P, Egan M. Identification of the Cystic Fibrosis Transmembrane Conductance Regulator Domains That Are Important for Interactions with ROMK2*. Journal Of Biological Chemistry 2000, 275: 16697-16701. PMID: 10748197, DOI: 10.1074/jbc.m910205199.Peer-Reviewed Original ResearchConceptsCystic fibrosis transmembrane conductance regulatorR domainCAMP-activated chloride channelFunctional chloride channelChloride channelsFibrosis transmembrane conductance regulatorFirst transmembrane domainTransmembrane domain 2Transmembrane domain 1Transmembrane conductance regulatorRegulator domainFold domainCFTR domainsTransmembrane domainCFTR regulationCFTR constructsConductance regulatorFirst nucleotideDomain 2Regulatory propertiesDomain 1Ion channelsXenopus oocytesPhosphorylationGlibenclamide sensitivity
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
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 recordingsCellsMechanismRegulator