2022
Update on Innate and Adaptive Immunity in Cystic Fibrosis
Bruscia E, Bonfield T. Update on Innate and Adaptive Immunity in Cystic Fibrosis. Clinics In Chest Medicine 2022, 43: 603-615. PMID: 36344069, DOI: 10.1016/j.ccm.2022.06.004.Peer-Reviewed Original ResearchConceptsChronic infectionCFTR modulator therapyRobust inflammatory responseCystic fibrosis pathophysiologyImmune dysregulationPatient ageExcessive inflammationModulator therapyLung microenvironmentLung infectionImmune mechanismsInflammatory responseAdaptive immunityMucociliary transportCF life expectancyCF lungCystic fibrosisInfectionLife expectancyImmunityCritical roleCurrent understandingMorbidityInflammationFibrosis
2020
Single-Cell Transcriptional Archetypes of Airway Inflammation in Cystic Fibrosis.
Schupp JC, Khanal S, Gomez JL, Sauler M, Adams TS, Chupp GL, Yan X, Poli S, Zhao Y, Montgomery RR, Rosas IO, Dela Cruz CS, Bruscia EM, Egan ME, Kaminski N, Britto CJ. Single-Cell Transcriptional Archetypes of Airway Inflammation in Cystic Fibrosis. American Journal Of Respiratory And Critical Care Medicine 2020, 202: 1419-1429. PMID: 32603604, PMCID: PMC7667912, DOI: 10.1164/rccm.202004-0991oc.Peer-Reviewed Original ResearchConceptsCF lung diseaseHealthy control subjectsImmune dysfunctionLung diseaseCystic fibrosisControl subjectsSputum cellsAbnormal chloride transportLung mononuclear phagocytesInnate immune dysfunctionDivergent clinical coursesImmune cell repertoireMonocyte-derived macrophagesCF monocytesAirway inflammationClinical courseProinflammatory featuresCell survival programInflammatory responseTissue injuryCell repertoireImmune functionTranscriptional profilesAlveolar macrophagesMononuclear phagocytesTargeting the Heme Oxygenase 1/Carbon Monoxide Pathway to Resolve Lung Hyper-Inflammation and Restore a Regulated Immune Response in Cystic Fibrosis
Di Pietro C, Öz HH, Murray TS, Bruscia EM. Targeting the Heme Oxygenase 1/Carbon Monoxide Pathway to Resolve Lung Hyper-Inflammation and Restore a Regulated Immune Response in Cystic Fibrosis. Frontiers In Pharmacology 2020, 11: 1059. PMID: 32760278, PMCID: PMC7372134, DOI: 10.3389/fphar.2020.01059.Peer-Reviewed Original ResearchCF lung diseaseCarbon monoxide pathwayCystic fibrosisImmune responseHO-1Lung diseaseInflammatory responseMonoxide pathwayBacterial infectionsHost defenseHO-1/CO pathwayOxidative stressDefective host defenseRegulated immune responseEndogenous HO-1Non-resolving inflammationBactericidal activityHO-1 activationHO-1 inductionCF lung tissueContinuous tissue damagePotential cellular mechanismsPersistent bacterial infectionsMonocytes/MΦsBactericidal mediators
2015
Innate and Adaptive Immunity in Cystic Fibrosis
Bruscia EM, Bonfield TL. Innate and Adaptive Immunity in Cystic Fibrosis. Clinics In Chest Medicine 2015, 37: 17-29. PMID: 26857765, DOI: 10.1016/j.ccm.2015.11.010.Peer-Reviewed Original ResearchConceptsImmune cellsCystic fibrosis lung diseaseLung tissue destructionAnti-inflammatory cytokinesRobust inflammatory responseElevated proinflammatoryImmune dysregulationUnresolved inflammationLung diseaseInflammatory responseLeading causeCF patientsTissue destructionAdaptive immunityCF lungCystic fibrosisHost defenseElevated numbersExocrine pancreasHyperinflammationLungTissue integrityCurrent understandingCellsProinflammatory
2008
Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/− Mice
Bruscia EM, Zhang PX, Ferreira E, Caputo C, Emerson JW, Tuck D, Krause DS, Egan ME. Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/− Mice. American Journal Of Respiratory Cell And Molecular Biology 2008, 40: 295-304. PMID: 18776130, PMCID: PMC2645527, DOI: 10.1165/rcmb.2008-0170oc.Peer-Reviewed Original ResearchConceptsExaggerated inflammatory responseExaggerated immune responseBone marrow-derived macrophagesIL-6Marrow-derived macrophagesCystic fibrosisCF miceKeratinocyte chemoattractantCytokine responsesInflammatory responseIL-1alphaImmune responseAlveolar macrophagesBronchoalveolar lavage fluidGranulocyte colony-stimulating factorNumber of neutrophilsChemoattractant protein-1CF lung diseaseElevated cytokine responseInnate immune systemImportant therapeutic targetCF mouse modelsPopulation of macrophagesColony-stimulating factorPseudomonas aeruginosa LPS