2021
Emerging technologies for cystic fibrosis transmembrane conductance regulator restoration in all people with CF
Egan ME. Emerging technologies for cystic fibrosis transmembrane conductance regulator restoration in all people with CF. Pediatric Pulmonology 2021, 56: s32-s39. PMID: 32681713, PMCID: PMC8114183, DOI: 10.1002/ppul.24965.Peer-Reviewed Original Research
2006
Engraftment of Donor‐Derived Epithelial Cells in Multiple Organs Following Bone Marrow Transplantation into Newborn Mice
Bruscia EM, Ziegler EC, Price JE, Weiner S, Egan ME, Krause DS. Engraftment of Donor‐Derived Epithelial Cells in Multiple Organs Following Bone Marrow Transplantation into Newborn Mice. Stem Cells 2006, 24: 2299-2308. PMID: 16794262, DOI: 10.1634/stemcells.2006-0166.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAnimals, NewbornBone Marrow TransplantationCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsFemaleFluorescent Antibody TechniqueHematopoietic Stem Cell TransplantationIn Situ Hybridization, FluorescenceMaleMiceMice, Inbred C57BLMice, Inbred StrainsMice, TransgenicRNA, MessengerY ChromosomeConceptsBone marrow-derived cellsMarrow-derived epithelial cellsBone marrow transplantationNewborn miceEpithelial cellsMarrow transplantationGI tractBone marrow-derived epithelial cellsDonor-derived epithelial cellsDoses of busulfanMarrow-derived cellsEngraftment of donorIrradiated adult recipientsMyeloablative regimenPreparative regimenAdult recipientsDifferent regimensEngrafted miceHematopoietic engraftmentGastrointestinal tractSurvival advantageTherapeutic benefitAdult miceMultiple organsBone marrowAssessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation
Bruscia EM, Price JE, Cheng EC, Weiner S, Caputo C, Ferreira EC, Egan ME, Krause DS. Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantation. Proceedings Of The National Academy Of Sciences Of The United States Of America 2006, 103: 2965-2970. PMID: 16481627, PMCID: PMC1413802, DOI: 10.1073/pnas.0510758103.Peer-Reviewed Original ResearchConceptsCftr-/- miceEpithelial cellsNasal epitheliumBM-derived cellsBone marrow transplantationWild-type BMAirway epithelial cellsCystic fibrosis transmembrane conductance regulator (CFTR) activityCystic fibrosis miceRare epithelial cellsCftr-null miceMarrow transplantationBM transplantationFibrosis miceRespiratory tractCFTR activityGI tractBone marrowGastrointestinalChloride secretionCFTR-dependent chloride secretionIndividual miceTransplantationDifferent dosesMice
2001
Effects of the Serine/Threonine Kinase SGK1 on the Epithelial Na+ Channel (ENaC) and CFTR: Implications for Cystic Fibrosis
Wagner C, Ott M, Klingel K, Beck S, Melzig J, Friedrich B, Wild K, Bröer S, Moschen I, Albers A, Waldegger S, Tümmler B, Egan M, Geibel J, Kandolf R, Lang F. Effects of the Serine/Threonine Kinase SGK1 on the Epithelial Na+ Channel (ENaC) and CFTR: Implications for Cystic Fibrosis. Cellular Physiology And Biochemistry 2001, 11: 209-218. PMID: 11509829, DOI: 10.1159/000051935.Peer-Reviewed Original ResearchMeSH Keywords1-Methyl-3-isobutylxanthineAmino Acid SubstitutionAnimalsBronchiCell LineCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsEpithelial Sodium ChannelsHumansIn Situ HybridizationLungMacrophages, AlveolarMutationOocytesPatch-Clamp TechniquesProtein Serine-Threonine KinasesPulmonary AlveoliRNA, ComplementaryRNA, MessengerSodiumSodium ChannelsXenopus laevisConceptsSerine/threonine kinase SGK1Lung tissueCystic fibrosisCF patientsKinase SGK1CF lung tissueXenopus oocytesLoss of CFTRLung epithelial cell lineCoexpression of CFTREffect of SGK1Pathophysiological factorsEpithelial cell lineRespiratory epitheliumLung phenotypeVariety of stimuliCl(-) secretionSGK1 expressionInhibitor amilorideInhibitory effectEpithelial cellsEnhanced expressionChannel ENaC.CFTR mutationsChannel activity
1999
A Novel Sulfonylurea Receptor Family Member Expressed in the Embryonic Drosophila Dorsal Vessel and Tracheal System*
Nasonkin I, Alikasifoglu A, Ambrose C, Cahill P, Cheng M, Sarniak A, Egan M, Thomas P. A Novel Sulfonylurea Receptor Family Member Expressed in the Embryonic Drosophila Dorsal Vessel and Tracheal System*. Journal Of Biological Chemistry 1999, 274: 29420-29425. PMID: 10506204, DOI: 10.1074/jbc.274.41.29420.Peer-Reviewed Original ResearchMeSH KeywordsAmino Acid SequenceAnimalsATP-Binding Cassette TransportersCloning, MolecularDrosophilaDrosophila ProteinsGene Expression Regulation, DevelopmentalGlyburideHumansIn Situ HybridizationMolecular Sequence DataOocytesPatch-Clamp TechniquesPhylogenyPotassium ChannelsPotassium Channels, Inwardly RectifyingReceptors, DrugRNA, MessengerSulfonylurea ReceptorsXenopus laevisConceptsDrosophila embryogenesisPotassium channel activityTracheal systemNovel Drosophila geneDrosophila dorsal vesselDorsal vesselABC transporter familyChannel activityReceptor family membersDrosophila genesGene duplicationPotassium channelsDrosophila systemTransporter familyGenetic approachesATP-sensitive potassium channel activityGenesFunctional studiesSulfonylurea receptorKir6.2 subunitEmbryogenesisATP-sensitive potassium channelsSURxSubunitsDistinctive sequenceCFTR is functionally active in GnRH-expressing GT1–7 hypothalamic neurons
Weyler R, Yurko-Mauro K, Rubenstein R, Kollen W, Reenstra W, Altschuler S, Egan M, Mulberg A. CFTR is functionally active in GnRH-expressing GT1–7 hypothalamic neurons. American Journal Of Physiology 1999, 277: c563-c571. PMID: 10484343, DOI: 10.1152/ajpcell.1999.277.3.c563.Peer-Reviewed Original ResearchConceptsGT1-7 hypothalamic neuronsHypothalamic neuronsHypothalamic neuronal cell lineGonadotropin-releasing hormoneGT1-7 cellsNeuronal cell linePreincubation of cellsGnRH secretionGT1-7Cystic fibrosisCFTR geneWestern blottingCystic fibrosis transmembrane conductance regulator (CFTR) geneCell linesGnRHDiverse manifestationsHuman brainNeuronsCAMP analogTransmembrane conductance regulator geneSexual differentiationExon 10CFTR activity