2017
Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophages
Di Pietro C, Zhang PX, O’Rourke T, Murray TS, Wang L, Britto CJ, Koff JL, Krause DS, Egan ME, Bruscia EM. Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophages. Scientific Reports 2017, 7: 10882. PMID: 28883468, PMCID: PMC5589856, DOI: 10.1038/s41598-017-11012-7.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsCell LineCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorCytoskeletal ProteinsDisease Models, AnimalMacrophage ActivationMacrophagesMicePhosphatidylinositol 3-KinasesProto-Oncogene Proteins c-aktPseudomonas aeruginosaPseudomonas InfectionsSignal TransductionToll-Like Receptor 4ConceptsCystic fibrosis transmembrane conductance regulatorPI3K/AktFibrosis transmembrane conductance regulatorTransmembrane conductance regulatorPI3K/Akt signalingConductance regulatorAnti-bacterial immune responseAkt signalingAltered localizationEzrinCystic fibrosis diseaseMφ activationAktProtein levelsFibrosis diseaseActivationImmune regulationPhagocytosisInductionDirect linkSignalingRegulatorImmune responseMΦMacrophages
2013
Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator
Villella VR, Esposito S, Bruscia EM, Vicinanza M, Cenci S, Guido S, Pettoello-Mantovani M, Carnuccio R, De Matteis MA, Luini A, Maiuri MC, Raia V, Kroemer G, Maiuri L. Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator. Cell Death & Differentiation 2013, 20: 1101-1115. PMID: 23686137, PMCID: PMC3705602, DOI: 10.1038/cdd.2013.46.Peer-Reviewed Original ResearchMeSH KeywordsAdaptor Proteins, Signal TransducingApoptosis Regulatory ProteinsBeclin-1BronchiCell LineCell MembraneCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsHumansMembrane ProteinsMutationPhosphoric Monoester HydrolasesProteostasis DeficienciesRab5 GTP-Binding ProteinsReceptors, TransferrinSequestosome-1 ProteinConceptsCFTR proteinBronchial epithelial cellsCFTR surface expressionSmall GTPase Rab5Cystic fibrosis transmembrane conductance regulatorFibrosis transmembrane conductance regulatorWild-type CFTRFunctional CFTR proteinSQSTM1/p62Transmembrane conductance regulatorPositive feed-forward loopPlasma membrane stabilityFeed-forward loopEpithelial cellsProteostasis regulationProtein traffickingProteostasis networkGTPase Rab5Rab5 effectorProteostasis regulatorsConformational diseasesCystic fibrosis transmembrane conductance regulator (CFTR) potentiatorRecycling defectsSQSTM1 accumulationUnexpected linkReduced Caveolin-1 Promotes Hyperinflammation due to Abnormal Heme Oxygenase-1 Localization in Lipopolysaccharide-Challenged Macrophages with Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator
Zhang PX, Murray TS, Villella VR, Ferrari E, Esposito S, D'Souza A, Raia V, Maiuri L, Krause DS, Egan ME, Bruscia EM. Reduced Caveolin-1 Promotes Hyperinflammation due to Abnormal Heme Oxygenase-1 Localization in Lipopolysaccharide-Challenged Macrophages with Dysfunctional Cystic Fibrosis Transmembrane Conductance Regulator. The Journal Of Immunology 2013, 190: 5196-5206. PMID: 23606537, PMCID: PMC3711148, DOI: 10.4049/jimmunol.1201607.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultAnimalsCaveolin 1Cells, CulturedChildChild, PreschoolCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorFemaleHeme Oxygenase-1HumansInflammationLipopolysaccharidesLung DiseasesMacrophagesMaleMembrane ProteinsMiceMice, KnockoutNasal PolypsReactive Oxygen SpeciesSignal TransductionToll-Like Receptor 4Young AdultConceptsCav-1 expressionHeme oxygenase-1Dysfunctional cystic fibrosis transmembrane conductance regulatorCystic fibrosis transmembrane conductance regulatorCell surfaceFibrosis transmembrane conductance regulatorProtein caveolin-1Cellular redox statusCell surface localizationCellular oxidative stateTransmembrane conductance regulatorHO-1 enzymePositive feed-forward loopCystic fibrosis macrophagesNegative regulatorCaveolin-1Conductance regulatorCell survivalHO-1 deliverySurface localizationRedox statusMΦ responsesHO-1/CO pathwayPathwayPotential targetTargeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect
Villella VR, Esposito S, Bruscia EM, Maiuri MC, Raia V, Kroemer G, Maiuri L. Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR Defect. Frontiers In Pharmacology 2013, 4: 1. PMID: 23346057, PMCID: PMC3549520, DOI: 10.3389/fphar.2013.00001.Peer-Reviewed Original ResearchCF transmembrane conductance regulatorIntracellular environmentMutant CF transmembrane conductance regulatorF508del-CFTR proteinBeclin-1CFTR channel activityTransmembrane conductance regulatorDefective CF transmembrane conductance regulatorDirect gene transferGeneral proteostasisProteostasis regulatorsConformational diseasesDisabled autophagyCFTR mutantsAutophagosome formationPlasma membraneConductance regulatorF508del-CFTRCommon deletion mutationsDeletion mutationsRecent pre-clinical evidenceIntracellular retentionGene transferCFTR defectProteostasis
2011
Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis
Bruscia EM, Zhang PX, Satoh A, Caputo C, Medzhitov R, Shenoy A, Egan ME, Krause DS. Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic Fibrosis. The Journal Of Immunology 2011, 186: 6990-6998. PMID: 21593379, PMCID: PMC3111054, DOI: 10.4049/jimmunol.1100396.Peer-Reviewed Original Research