2024
Chronic lung inflammation disrupts the quiescent state of hematopoietic stem cells in a cystic fibrosis mouse model
Braga C, Mancuso R, Thompson E, Oez H, Gudneppanavar R, Zhang P, Huang P, Murray T, Egan M, Krause D, Bruscia E. Chronic lung inflammation disrupts the quiescent state of hematopoietic stem cells in a cystic fibrosis mouse model. The Journal Of Immunology 2024, 212: 0062_6002-0062_6002. DOI: 10.4049/jimmunol.212.supp.0062.6002.Peer-Reviewed Original ResearchHematopoietic stem cellsChronic lung inflammationLung inflammationCystic fibrosisBone marrowQuiescent state of HSCsProgression of CF lung diseaseResponse to airway infectionWT hematopoietic stem cellsExpansion of HSCsMultipotent progenitorsCystic fibrosis mouse modelStem cellsCF lung diseasePathways associated with proliferationNeutrophilic lung inflammationPro-inflammatory signatureFibrosis mouse modelATAC-sequencing analysisAirway infectionBM cellsMyeloid lineageLung diseaseMouse modelInflammation
2022
Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis
Öz H, Cheng E, Di Pietro C, Tebaldi T, Biancon G, Zeiss C, Zhang P, Huang P, Esquibies S, Britto C, Schupp J, Murray T, Halene S, Krause D, Egan M, Bruscia E. Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosis. Cell Reports 2022, 41: 111797. PMID: 36516754, PMCID: PMC9833830, DOI: 10.1016/j.celrep.2022.111797.Peer-Reviewed Original ResearchConceptsC motif chemokine receptor 2Monocytes/macrophagesLung tissue damageCystic fibrosisTissue damageCF lungPulmonary neutrophilic inflammationPro-inflammatory environmentChemokine receptor 2CF lung diseaseNumber of monocytesSpecific therapeutic agentsGrowth factor βCF transmembrane conductance regulatorLung hyperinflammationLung neutrophiliaNeutrophilic inflammationNeutrophil inflammationInflammation contributesLung damageNeutrophil recruitmentLung diseaseLung tissueReceptor 2Therapeutic target
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
2012
Innate immunity in cystic fibrosis lung disease
Hartl D, Gaggar A, Bruscia E, Hector A, Marcos V, Jung A, Greene C, McElvaney G, Mall M, Döring G. Innate immunity in cystic fibrosis lung disease. Journal Of Cystic Fibrosis 2012, 11: 363-382. PMID: 22917571, DOI: 10.1016/j.jcf.2012.07.003.Peer-Reviewed Original ResearchConceptsLung diseaseCF lung diseaseInnate immunityChronic infective lung diseaseNovel immune-targeted therapiesCystic fibrosis lung diseasePulmonary immune responseChronic lung diseaseImmune-targeted therapiesPro-inflammatory cascadeInfective lung diseaseInnate immune regulationInnate immune systemCystic fibrosis patientsPotential clinical relevanceEpithelial dysfunctionLeukocyte recruitmentImmune regulationImmune responseAdaptive immunityClinical relevanceFibrosis patientsImmune systemDiseaseImmunity
2002
Towards the pharmacogenomics of cystic fibrosis
Sangiuolo F, DApice M, Bruscia E, Lucidi V, Novelli G. Towards the pharmacogenomics of cystic fibrosis. Pharmacogenomics 2002, 3: 75-87. PMID: 11966405, DOI: 10.1517/14622416.3.1.75.Peer-Reviewed Original ResearchConceptsCystic fibrosisPancreatic insufficiencyChronic obstructive lung diseaseRelated clinical diseasesObstructive lung diseaseElevated sweat chloride concentrationsExocrine pancreatic insufficiencySweat chloride concentrationCongenital bilateral absenceMultiorgan diseaseChronic pancreatitisClinical symptomsLung diseaseDrug therapyClinical diseaseClinical overlapRecessive genetic diseaseTherapeutic questionsBilateral absenceDrug efficacyDiseaseVas deferensTherapy approachesFibrosisSymptoms