Emanuela Bruscia, PhD
Research & Publications
Biography
News
Research Summary
My research focuses on understanding the molecular mechanisms behind the development of lung disease in patients with cystic fibrosis (CF). For over 16 years, we have studied how monocytes and macrophages contribute to CF's dysregulated innate immune response. We investigate the mechanism/s by which the dysregulated activity of these immune cells contributes to the overwhelming lung inflammation, the weakened host defense against certain microorganisms, and the altered lung tissue repair processes that characterize CF lung disease. Ultimately, we aim to identify mechanism-based therapeutic targets that, in conjunction with the current CFTR modulator therapies, will help prevent inflammation and progressive lung tissue deterioration in individuals with CF.
Specialized Terms: Cystic Fibrosis; Lung inflammation; Macrophages
Coauthors
Research Interests
Cystic Fibrosis; Lung; Macrophages; Pediatrics; Respiration Disorders; Cystic Fibrosis Transmembrane Conductance Regulator
Research Image
Cystic Fibrosis and dysregulated immune response
Selected Publications
- 93 Persistent airway infections in cystic fibrosis–like mouse models correlate with phenotypic changes of clinical Pseudomonas aeruginosa isolatesOez H, Braga C, Laurence E, Gudneppanavar R, Huang P, Zhang P, Egan M, Bruscia E, Murray T. 93 Persistent airway infections in cystic fibrosis–like mouse models correlate with phenotypic changes of clinical Pseudomonas aeruginosa isolates. Journal Of Cystic Fibrosis 2023, 22: s48. DOI: 10.1016/s1569-1993(23)01028-7.
- 359 Distinct IL-10low IFN-γhigh signature is associated with IgG versus IgE response to peanut allergen exposure in CFTR–/– miceEmmenegger M, Hoyt L, Gudneppanavar R, Eisenbarth S, Egan M, Bruscia E, Aguzzi A. 359 Distinct IL-10low IFN-γhigh signature is associated with IgG versus IgE response to peanut allergen exposure in CFTR–/– mice. Journal Of Cystic Fibrosis 2023, 22: s182. DOI: 10.1016/s1569-1993(23)01286-9.
- 361 Chronic lung inflammation abrogates quiescent state of hematopoietic stem cells in a cystic fibrosis mouse modelBraga C, Mancuso R, Thompson E, Oez H, Gudneppanavar R, Zhang P, Huang P, Murray T, Egan M, Krause D, Bruscia E. 361 Chronic lung inflammation abrogates quiescent state of hematopoietic stem cells in a cystic fibrosis mouse model. Journal Of Cystic Fibrosis 2023, 22: s183. DOI: 10.1016/s1569-1993(23)01288-2.
- 276 Delivery of CFTR mRNA using polymeric nanoparticles in a cystic fibrosis mouse modelGarrison A, Suberi A, Browne J, Eaton D, Akhtar L, Kizilirmak T, Bruscia E, Piotrowski-Daspit A, Saltzman W, Egan M. 276 Delivery of CFTR mRNA using polymeric nanoparticles in a cystic fibrosis mouse model. Journal Of Cystic Fibrosis 2023, 22: s140. DOI: 10.1016/s1569-1993(23)01204-3.
- 220 Ezrin is required to maintain the number and function of tissue resident alveolar macrophages in cystic fibrosis during infectionGudneppanavar R, Di Pietro C, Oez H, Zhang P, Huang P, Britto-Leon C, Krause D, Egan M, Murray T, Bruscia E. 220 Ezrin is required to maintain the number and function of tissue resident alveolar macrophages in cystic fibrosis during infection. Journal Of Cystic Fibrosis 2023, 22: s113. DOI: 10.1016/s1569-1993(23)01150-5.
- 150 Optimizing workflowto understand the impact of CFTR dysfunction on airway immune cell transcriptomes in early disease pathogenesisKizilirmak T, Garrison A, Yin H, Bruscia E, Egan M, Britto-Leon C. 150 Optimizing workflowto understand the impact of CFTR dysfunction on airway immune cell transcriptomes in early disease pathogenesis. Journal Of Cystic Fibrosis 2023, 22: s79-s80. DOI: 10.1016/s1569-1993(23)01082-2.
- The effects of elexafactor/tezafactor/ivacaftor beyond the epithelium: spurring macrophages to fight infections.Bruscia E. The effects of elexafactor/tezafactor/ivacaftor beyond the epithelium: spurring macrophages to fight infections. European Respiratory Journal 2023, 61: 2300216. PMID: 37003613, DOI: 10.1183/13993003.00216-2023.
- Update on Innate and Adaptive Immunity in Cystic FibrosisBruscia 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.
- Recruited monocytes/macrophages drive pulmonary neutrophilic inflammation and irreversible lung tissue remodeling in cystic fibrosisÖz 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.
- Human neutrophil development and functionality are enabled in a humanized mouse modelZheng Y, Sefik E, Astle J, Karatepe K, Öz HH, Solis AG, Jackson R, Luo HR, Bruscia EM, Halene S, Shan L, Flavell RA. Human neutrophil development and functionality are enabled in a humanized mouse model. Proceedings Of The National Academy Of Sciences Of The United States Of America 2022, 119: e2121077119. PMID: 36269862, PMCID: PMC9618085, DOI: 10.1073/pnas.2121077119.
- 455 Impaired recruitment of C-C motif chemokine receptor 2-positive monocytes does not compromise host defense against pulmonary Pseudomonas aeruginosa infectionsÖz H, Di Pietro C, Rodrigues G, Zhang P, Huang P, Egan M, Murray T, Bruscia E. 455 Impaired recruitment of C-C motif chemokine receptor 2-positive monocytes does not compromise host defense against pulmonary Pseudomonas aeruginosa infections. Journal Of Cystic Fibrosis 2022, 21: s260. DOI: 10.1016/s1569-1993(22)01145-6.
- 674 Loss of functional cystic fibrosis transmembrane conductance regulator leads to a calpain-dependent degradation of ezrin in activated cystic fibrosis macrophagesDi Pietro C, Öz H, Rodrigues G, Huang P, Zhang P, Murray T, Egan M, Bruscia E. 674 Loss of functional cystic fibrosis transmembrane conductance regulator leads to a calpain-dependent degradation of ezrin in activated cystic fibrosis macrophages. Journal Of Cystic Fibrosis 2022, 21: s369. DOI: 10.1016/s1569-1993(22)01364-9.
- 491 Pel overexpression by Pseudomonas aeruginosa reduces induction of heme-oxygenase 1 in macrophagesRodrigues G, Di Pietro C, Huang P, Öz H, Bruscia E, Murray T. 491 Pel overexpression by Pseudomonas aeruginosa reduces induction of heme-oxygenase 1 in macrophages. Journal Of Cystic Fibrosis 2022, 21: s278. DOI: 10.1016/s1569-1993(22)01181-x.
- Emerging Concepts in Defective Macrophage Phagocytosis in Cystic FibrosisJaganathan D, Bruscia EM, Kopp BT. Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis. International Journal Of Molecular Sciences 2022, 23: 7750. PMID: 35887098, PMCID: PMC9319215, DOI: 10.3390/ijms23147750.
- Gene therapy applications to transfusion medicineTabibi S, Gehrie E, Bruscia E, Krause D. Gene therapy applications to transfusion medicine. 2022, 642-647. DOI: 10.1002/9781119719809.ch56.
- Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosisDi Pietro C, Öz HH, Zhang PX, Cheng EC, Martis V, Bonfield TL, Kelley TJ, Jubin R, Abuchowski A, Krause DS, Egan ME, Murray TS, Bruscia EM. Recruitment of monocytes primed to express heme oxygenase-1 ameliorates pathological lung inflammation in cystic fibrosis. Experimental & Molecular Medicine 2022, 54: 639-652. PMID: 35581352, PMCID: PMC9166813, DOI: 10.1038/s12276-022-00770-8.
- 558: Carbon monoxide–based therapy primes macrophages to express HO-1 and to resolve lung hyper-inflammation in cystic fibrosisPietro C, Öz H, Zhang P, Cheng E, Martis V, Bonfield T, Kelley T, Jubin R, Abuchowski A, Krause D, Egan M, Murray T, Bruscia E. 558: Carbon monoxide–based therapy primes macrophages to express HO-1 and to resolve lung hyper-inflammation in cystic fibrosis. Journal Of Cystic Fibrosis 2021, 20: s263-s264. DOI: 10.1016/s1569-1993(21)01981-0.
- 473: Impairing the recruitment of inflammatory monocytes to CF lungs does not weaken host defense against pulmonary Pseudomonas aeruginosa infectionsÖz H, Di Pietro C, Cheng E, Zhang P, Huang P, Murray T, Egan M, Bruscia E. 473: Impairing the recruitment of inflammatory monocytes to CF lungs does not weaken host defense against pulmonary Pseudomonas aeruginosa infections. Journal Of Cystic Fibrosis 2021, 20: s223. DOI: 10.1016/s1569-1993(21)01897-x.
- Combined liver–cytokine humanization comes to the rescue of circulating human red blood cellsSong Y, Shan L, Gbyli R, Liu W, Strowig T, Patel A, Fu X, Wang X, Xu ML, Gao Y, Qin A, Bruscia EM, Tebaldi T, Biancon G, Mamillapalli P, Urbonas D, Eynon E, Gonzalez DG, Chen J, Krause DS, Alderman J, Halene S, Flavell RA. Combined liver–cytokine humanization comes to the rescue of circulating human red blood cells. Science 2021, 371: 1019-1025. PMID: 33674488, PMCID: PMC8292008, DOI: 10.1126/science.abe2485.
- 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.
- Reconstruction of Sickle Cell Disease with Circulating Sickling Red Blood Cells in Novel Humanized Cytokines and Liver Mistrg MiceSong Y, Gbyli R, Shan L, Liu W, Gao Y, Patel A, Fu X, Wang X, Xu M, Qin A, Bruscia E, Tebaldi T, Biancon G, Mamillapalli P, Urbonas D, Gonzales D, Krause D, Alderman J, Flavell R, Halene S. Reconstruction of Sickle Cell Disease with Circulating Sickling Red Blood Cells in Novel Humanized Cytokines and Liver Mistrg Mice. Blood 2020, 136: 29-30. DOI: 10.1182/blood-2020-141603.
- Targeting the Heme Oxygenase 1/Carbon Monoxide Pathway to Resolve Lung Hyper-Inflammation and Restore a Regulated Immune Response in Cystic FibrosisDi 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.
- Macrophages in Chronic CF Lung Inflammation, Resolution and RemodelingOz H, Di Pietro C, Cheng E, Zhang P, Nguyen R, Barone C, Krause D, Egan M, Bruscia E. Macrophages in Chronic CF Lung Inflammation, Resolution and Remodeling. 2019, a6186-a6186. DOI: 10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a6186.
- Targeting the HO-1/CO Pathway with Sanguinate: Toward Development of a Novel Anti-inflammatory Therapy for Cystic Fibrosis (CF) Lung DiseaseDi Pietro C, Oz H, Martis V, Zhang P, Jubin R, Abuchowski A, Krause D, Egan M, Murray T, Bruscia E. Targeting the HO-1/CO Pathway with Sanguinate: Toward Development of a Novel Anti-inflammatory Therapy for Cystic Fibrosis (CF) Lung Disease. 2019, a6187-a6187. DOI: 10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a6187.
- Electric Cell- Substrate Impedance Sensing Reveals Defective Permeability in Cystic Fibrosis Bronchial Epithelial CellsSendon C, DiPietro C, Oez H, Barone C, Pierce R, Egan M, Bruscia E. Electric Cell- Substrate Impedance Sensing Reveals Defective Permeability in Cystic Fibrosis Bronchial Epithelial Cells. 2019, a6192-a6192. DOI: 10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a6192.
- A highly efficient and faithful MDS patient-derived xenotransplantation model for pre-clinical studiesSong Y, Rongvaux A, Taylor A, Jiang T, Tebaldi T, Balasubramanian K, Bagale A, Terzi YK, Gbyli R, Wang X, Fu X, Gao Y, Zhao J, Podoltsev N, Xu M, Neparidze N, Wong E, Torres R, Bruscia EM, Kluger Y, Manz MG, Flavell RA, Halene S. A highly efficient and faithful MDS patient-derived xenotransplantation model for pre-clinical studies. Nature Communications 2019, 10: 366. PMID: 30664659, PMCID: PMC6341122, DOI: 10.1038/s41467-018-08166-x.
- Surfactant protein C dampens inflammation by decreasing JAK/STAT activation during lung repairJin H, Ciechanowicz AK, Kaplan AR, Wang L, Zhang P, Lu YC, Tobin RE, Tobin BA, Cohn L, Zeiss CJ, Lee PJ, Bruscia EM, Krause DS. Surfactant protein C dampens inflammation by decreasing JAK/STAT activation during lung repair. American Journal Of Physiology - Lung Cellular And Molecular Physiology 2018, 314: l882-l892. PMID: 29345196, PMCID: PMC6008135, DOI: 10.1152/ajplung.00418.2017.
- Ezrin links CFTR to TLR4 signaling to orchestrate anti-bacterial immune response in macrophagesDi 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.
- Cellular Innate Immunity: An Old Game with New PlayersGasteiger G, D'Osualdo A, Schubert DA, Weber A, Bruscia EM, Hartl D. Cellular Innate Immunity: An Old Game with New Players. Journal Of Innate Immunity 2016, 9: 111-125. PMID: 28006777, PMCID: PMC6738785, DOI: 10.1159/000453397.
- Cystic Fibrosis Lung Immunity: The Role of the MacrophageBruscia EM, Bonfield TL. Cystic Fibrosis Lung Immunity: The Role of the Macrophage. Journal Of Innate Immunity 2016, 8: 550-563. PMID: 27336915, PMCID: PMC5089923, DOI: 10.1159/000446825.
- Gene therapy applications to transfusion medicineGehrie E, Bersenev A, Bruscia E, Krause D, Schulz W. Gene therapy applications to transfusion medicine. 2016, 452-455. DOI: 10.1002/9781119013020.ch38.
- Increased susceptibility of Cftr−/− mice to LPS-induced lung remodelingBruscia E, Zhang P, Barone C, Scholte BJ, Homer R, Krause D, Egan ME. Increased susceptibility of Cftr−/− mice to LPS-induced lung remodeling. American Journal Of Physiology - Lung Cellular And Molecular Physiology 2016, 310: l711-l719. PMID: 26851259, PMCID: PMC4836110, DOI: 10.1152/ajplung.00284.2015.
- Innate and Adaptive Immunity in Cystic FibrosisBruscia 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.
- Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammationZhang PX, Cheng J, Zou S, D'Souza AD, Koff JL, Lu J, Lee PJ, Krause DS, Egan ME, Bruscia EM. Pharmacological modulation of the AKT/microRNA-199a-5p/CAV1 pathway ameliorates cystic fibrosis lung hyper-inflammation. Nature Communications 2015, 6: 6221. PMID: 25665524, PMCID: PMC4324503, DOI: 10.1038/ncomms7221.
- SRF is required for neutrophil migration in response to inflammationTaylor A, Tang W, Bruscia EM, Zhang PX, Lin A, Gaines P, Wu D, Halene S. SRF is required for neutrophil migration in response to inflammation. Blood 2014, 123: 3027-3036. PMID: 24574460, PMCID: PMC4014845, DOI: 10.1182/blood-2013-06-507582.
- Very Small Embryonic‐Like Stem Cells from the Murine Bone Marrow Differentiate into Epithelial Cells of the LungKassmer SH, Jin H, Zhang PX, Bruscia EM, Heydari K, Lee JH, Kim CF, Kassmer SH, Krause DS. Very Small Embryonic‐Like Stem Cells from the Murine Bone Marrow Differentiate into Epithelial Cells of the Lung. Stem Cells 2013, 31: 2759-2766. PMID: 23681901, PMCID: PMC4536826, DOI: 10.1002/stem.1413.
- Srf Is Required For Neutrophil Migration In Response To InflammationTaylor A, Tang W, Bruscia E, Zhang P, Lin A, Gaines P, Vu L, Wu D, Halene S. Srf Is Required For Neutrophil Migration In Response To Inflammation. Blood 2013, 122: 319-319. DOI: 10.1182/blood.v122.21.319.319.
- Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulatorVillella 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.
- Reduced Caveolin-1 Promotes Hyperinflammation due to Abnormal Heme Oxygenase-1 Localization in Lipopolysaccharide-Challenged Macrophages with Dysfunctional Cystic Fibrosis Transmembrane Conductance RegulatorZhang 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.
- Targeting the Intracellular Environment in Cystic Fibrosis: Restoring Autophagy as a Novel Strategy to Circumvent the CFTR DefectVillella 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.
- Innate immunity in cystic fibrosis lung diseaseHartl 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.
- Nebulized Hyaluronan Ameliorates lung inflammation in cystic fibrosis miceGavina M, Luciani A, Villella VR, Esposito S, Ferrari E, Bressani I, Casale A, Bruscia EM, Maiuri L, Raia V. Nebulized Hyaluronan Ameliorates lung inflammation in cystic fibrosis mice. Pediatric Pulmonology 2012, 48: 761-771. PMID: 22825912, DOI: 10.1002/ppul.22637.
- 52 Nebulized hyaluronan ameliorates lung inflammation in cystic fibrosis (CF) miceGavina M, Luciani A, Villella V, Esposito S, Ferrari E, Casale A, De Santis S, Bruscia E, Maiuri L, Raia V, Tosco A. 52 Nebulized hyaluronan ameliorates lung inflammation in cystic fibrosis (CF) mice. Journal Of Cystic Fibrosis 2012, 11: s69. DOI: 10.1016/s1569-1993(12)60221-5.
- Calcium Modulation Decreases Chloride Efflux And Cytokine Secretion In Activated Cystic Fibrosis Murine MacrophagesShenoy A, Kopic S, Bruscia E, Caputo C, Geibel J, Egan M. Calcium Modulation Decreases Chloride Efflux And Cytokine Secretion In Activated Cystic Fibrosis Murine Macrophages. 2012, a5272-a5272. DOI: 10.1164/ajrccm-conference.2012.185.1_meetingabstracts.a5272.
- The Carbon Monoxide Releasing Molecule CORM-2 Attenuates Pseudomonas aeruginosa Biofilm FormationMurray TS, Okegbe C, Gao Y, Kazmierczak BI, Motterlini R, Dietrich LE, Bruscia EM. The Carbon Monoxide Releasing Molecule CORM-2 Attenuates Pseudomonas aeruginosa Biofilm Formation. PLOS ONE 2012, 7: e35499. PMID: 22563385, PMCID: PMC3338523, DOI: 10.1371/journal.pone.0035499.
- Nonhematopoietic Cells are the Primary Source of Bone Marrow‐Derived Lung Epithelial CellsKassmer SH, Bruscia EM, Zhang P, Krause DS. Nonhematopoietic Cells are the Primary Source of Bone Marrow‐Derived Lung Epithelial Cells. Stem Cells 2012, 30: 491-499. PMID: 22162244, PMCID: PMC3725285, DOI: 10.1002/stem.1003.
- Abnormal Trafficking and Degradation of TLR4 Underlie the Elevated Inflammatory Response in Cystic FibrosisBruscia 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.
- Bone Marrow Derived Lung Epithelial Cells Are Derived Predominantly From Nonhematopoietic Cells.Kassmer S, Bruscia E, Zhang P, Krause D. Bone Marrow Derived Lung Epithelial Cells Are Derived Predominantly From Nonhematopoietic Cells. Blood 2010, 116: 2615-2615. DOI: 10.1182/blood.v116.21.2615.2615.
- Role for MKL1 in megakaryocytic maturationCheng EC, Luo Q, Bruscia EM, Renda MJ, Troy JA, Massaro SA, Tuck D, Schulz V, Mane SM, Berliner N, Sun Y, Morris SW, Qiu C, Krause DS. Role for MKL1 in megakaryocytic maturation. Blood 2009, 113: 2826-2834. PMID: 19136660, PMCID: PMC2661865, DOI: 10.1182/blood-2008-09-180596.
- Gene Therapy in Transfusion MedicineBruscia E, Krause D. Gene Therapy in Transfusion Medicine. 2009, 936-949. DOI: 10.1002/9781444303513.ch60.
- Macrophages Directly Contribute to the Exaggerated Inflammatory Response in Cystic Fibrosis Transmembrane Conductance Regulator−/− MiceBruscia 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.
- Rectal Potential Difference and the Functional Expression of CFTR in the Gastrointestinal Epithelia in Cystic Fibrosis Mouse ModelsWeiner 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.
- Cftr gene targeting in mouse embryonic stem cells mediated by Small Fragment Homologous Replacement (SFHR).Sangiuolo F, Scaldaferri ML, Filareto A, Spitalieri P, Guerra L, Favia M, Caroppo R, Mango R, Bruscia E, Gruenert DC, Casavola V, De Felici M, Novelli G. Cftr gene targeting in mouse embryonic stem cells mediated by Small Fragment Homologous Replacement (SFHR). Frontiers In Bioscience-Landmark 2008, 13: 2989-99. PMID: 17981772, PMCID: PMC3725395, DOI: 10.2741/2904.
- Engraftment of Donor‐Derived Epithelial Cells in Multiple Organs Following Bone Marrow Transplantation into Newborn MiceBruscia 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.
- Assessment of cystic fibrosis transmembrane conductance regulator (CFTR) activity in CFTR-null mice after bone marrow transplantationBruscia 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.
- 1054. Hematopoietic and Non-Hematopoietic Engraftment after Bone Marrow Transplantation in Newborn MiceBruscia E, Price J, Ziegler E, Krause D. 1054. Hematopoietic and Non-Hematopoietic Engraftment after Bone Marrow Transplantation in Newborn Mice. Molecular Therapy 2006, 13: s404. DOI: 10.1016/j.ymthe.2006.08.1150.
- 82. Cftr Gene Targeting in Murine ES Cells Mediated by the SFHR TechniqueSangiuolo F, Favia M, Guerra L, Filareto A, Scaldaferri M, Caroppo R, Spitalieri P, Mango R, Bruscia E, Gruenert D, Casavola V, De Felici M, Novelli G. 82. Cftr Gene Targeting in Murine ES Cells Mediated by the SFHR Technique. Molecular Therapy 2006, 13: s34-s35. DOI: 10.1016/j.ymthe.2006.08.100.
- In Vitro Restoration of Functional SMN Protein in Human Trophoblast Cells Affected by Spinal Muscular Atrophy by Small Fragment Homologous ReplacementSangiuolo F, Filareto A, Spitalieri P, Scaldaferri ML, Mango R, Bruscia E, Citro G, Brunetti E, De Felici M, Novelli G. In Vitro Restoration of Functional SMN Protein in Human Trophoblast Cells Affected by Spinal Muscular Atrophy by Small Fragment Homologous Replacement. Human Gene Therapy 2005, 16: 869-880. PMID: 16000068, DOI: 10.1089/hum.2005.16.869.
- In Vitro Restoration of Functional SMN Protein in Human Trophoblast Cells Affected by Spinal Muscular Atrophy by Small Fragment Homologous ReplacementSangiuolo F, Filareto A, Spitalieri P, Scaldaferri M, Mango R, Bruscia E, Citro G, Brunetti E, De Felici M, Novelli G. In Vitro Restoration of Functional SMN Protein in Human Trophoblast Cells Affected by Spinal Muscular Atrophy by Small Fragment Homologous Replacement. Human Gene Therapy 2005, 0: 050701034702010. DOI: 10.1089/hum.2005.16.ft-84.
- Engraftment of bone marrow-derived epithelial cellsVan Arnam JS, Herzog E, Grove J, Bruscia E, Ziegler E, Swenson S, Krause DS. Engraftment of bone marrow-derived epithelial cells. Stem Cell Reviews And Reports 2005, 1: 21-27. PMID: 17132871, DOI: 10.1385/scr:1:1:021.
- Lack of a Fusion Requirement for Development of Bone Marrow-Derived EpitheliaHarris RG, Herzog EL, Bruscia EM, Grove JE, Van Arnam JS, Krause DS. Lack of a Fusion Requirement for Development of Bone Marrow-Derived Epithelia. Science 2004, 305: 90-93. PMID: 15232107, DOI: 10.1126/science.1098925.
- Plasticity of Bone Marrow–Derived Stem CellsGrove JE, Bruscia E, Krause DS. Plasticity of Bone Marrow–Derived Stem Cells. Stem Cells 2004, 22: 487-500. PMID: 15277695, DOI: 10.1634/stemcells.22-4-487.
- Letter to the EditorsGruenert DC, Kunzelmann K, Novelli G, Colosimo A, Kapsa R, Bruscia E. Letter to the Editors. Nucleic Acid Therapeutics 2004, 14: 157-158. PMID: 15294078, DOI: 10.1089/1545457041526272.
- Sequence-specific modification of genomic DNA by small DNA fragmentsGruenert DC, Bruscia E, Novelli G, Colosimo A, Dallapiccola B, Sangiuolo F, Goncz KK. Sequence-specific modification of genomic DNA by small DNA fragments. Journal Of Clinical Investigation 2003, 112: 637-641. PMID: 12952908, PMCID: PMC182219, DOI: 10.1172/jci19773.
- In vitrocorrection of cystic fibrosis epithelial cell lines by small fragment homologous replacement (SFHR) techniqueSangiuolo F, Bruscia E, Serafino A, Nardone AM, Bonifazi E, Lais M, Gruenert DC, Novelli G. In vitrocorrection of cystic fibrosis epithelial cell lines by small fragment homologous replacement (SFHR) technique. BMC Medical Genomics 2002, 3: 8. PMID: 12243649, PMCID: PMC130050, DOI: 10.1186/1471-2350-3-8.
- Isolation of CF cell lines corrected at ΔF508-CFTR locus by SFHR-mediated targetingBruscia E, Sangiuolo F, Sinibaldi P, Goncz KK, Novelli G, Gruenert DC. Isolation of CF cell lines corrected at ΔF508-CFTR locus by SFHR-mediated targeting. Gene Therapy 2002, 9: 683-685. PMID: 12032687, DOI: 10.1038/sj.gt.3301741.
- Towards the pharmacogenomics of cystic fibrosisSangiuolo 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.
- Fine mapping of a distinctive autosomal dominant vacuolar neuromyopathy using 11 novel microsatellite markers from chromosome band 19p13.3Sangiuolo F, Bruscia E, Capon F, Servidei S, Dallapiccola B, Novelli G. Fine mapping of a distinctive autosomal dominant vacuolar neuromyopathy using 11 novel microsatellite markers from chromosome band 19p13.3. European Journal Of Human Genetics 2000, 8: 809-812. PMID: 11039585, DOI: 10.1038/sj.ejhg.5200547.
- Gene transfection efficiency of tracheal epithelial cells by DC-Chol–DOPE/DNA complexesColosimo A, Serafino A, Sangiuolo F, Di Sario S, Bruscia E, Amicucci P, Novelli G, Dallapiccola B, Mossa G. Gene transfection efficiency of tracheal epithelial cells by DC-Chol–DOPE/DNA complexes. Biochimica Et Biophysica Acta 1999, 1419: 186-194. PMID: 10407070, DOI: 10.1016/s0005-2736(99)00067-x.
- Genomic structure, promoter characterisation and mutational analysis of the S100A7 gene: exclusion of a candidate for familial psoriasis susceptibilitySemprini S, Capon F, Bovolenta S, Bruscia E, Pizzuti A, Fabrizi G, Schietroma C, Zambruno G, Dallapiccola B, Novelli G. Genomic structure, promoter characterisation and mutational analysis of the S100A7 gene: exclusion of a candidate for familial psoriasis susceptibility. Human Genetics 1999, 104: 130-134. PMID: 10190323, DOI: 10.1007/s004390050925.