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