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
1998
[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 mutation