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Marie Egan, MD

Professor of Pediatrics (Respiratory) and of Cellular And Molecular Physiology; Director, Cystic Fibrosis Center; Vice Chair for Research, Department of Pediatrics

Research & Publications
Biography
News

Research Summary

Dr Egan has demonstrated a long-term commitment to her overall goal, which is to elucidate the mechanisms that underlie cystic fibrosis (CF) related lung disease, in order to develop innovative and novel therapies and ultimately lengthen the life of patients with this lethal disorder. Dr. Egan’s primary research interest is to understand the function of CFTR in health and disease in epithelial and immune cells.

Extensive Research Description

Dr. Egan’s primary research interest is to understand the regulation of ion transport across the airway epithelia in health and disease. Transepithelial ion transport is responsible for maintaining the airway surface fluid, i.e. the periciliary fluid layer, which controls mucociliary clearance. Abnormalities in the ion channels and regulators of these channels can alter mucociliary clearance, leading to retained secretions, mucus plugging, infection, and lung destruction, as seen in cystic fibrosis. In CF, it is the abnormal function of the cystic fibrosis transmembrane conductance regulator (CFTR), a multifunctional protein encoded by the gene that is affected in cystic fibrosis (CF) that underlies the abnormal ion transport in affected organs.

The Egan lab uses a variety of electrophysiologic techniques to examine how CFTR expression affects transepithelial ion transport in airway epithelial cells. They have shown that CFTR can modulate other ion channels and, as its name implies, act as a conductance regulator. In addition, the laboratory is interested in examining how mutations in CFTR affect its ability to function. Lastly, the epithelium interacts with the airway microenvironment and primary immune cells to propagate disease. Dr. Egan has shown that CFTR functions in primary immune cells and this function is altered in CF contributing to disease.

The Egan lab has worked collaboratively to bring forth innovative platforms that could have great impact on CF patients such as gene editing.

Specialized Terms: Cystic fibrosis clinical studies; Cystic fibrosis basic science research (ion transport, Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) function) ion transport; Cystic fibrosis translational research studies (strategies to bypass or correct the basic defect) gene editing, immune response , pediatrics

Coauthors

Research Interests

Bacterial Infections; Cystic Fibrosis; Epithelial Cells; Genetic Code; Immunity, Innate; Inflammation; Lung; Macrophages; Microbiology; Pediatrics; Physiology; Pseudomonas aeruginosa; Clinical Trial; Macrophages, Alveolar; Cystic Fibrosis Transmembrane Conductance Regulator; Peptide Nucleic Acids; Nanoparticles; Adaptive Immunity; Translational Research, Biomedical; Airway Management; Gene Editing

Selected Publications

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.
In vivo correction of cystic fibrosis mediated by PNA nanoparticlesPiotrowski-Daspit AS, Barone C, Lin CY, Deng Y, Wu D, Binns TC, Xu E, Ricciardi AS, Putman R, Garrison A, Nguyen R, Gupta A, Fan R, Glazer PM, Saltzman WM, Egan ME. In vivo correction of cystic fibrosis mediated by PNA nanoparticles. Science Advances 2022, 8: eabo0522. PMID: 36197984, PMCID: PMC9534507, DOI: 10.1126/sciadv.abo0522.
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.
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.
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.
Surface conjugation of antibodies improves nanoparticle uptake in bronchial epithelial cellsLuks VL, Mandl H, DiRito J, Barone C, Freedman-Weiss MR, Ricciardi AS, Tietjen GG, Egan ME, Saltzman WM, Stitelman DH. Surface conjugation of antibodies improves nanoparticle uptake in bronchial epithelial cells. PLOS ONE 2022, 17: e0266218. PMID: 35385514, PMCID: PMC8986008, DOI: 10.1371/journal.pone.0266218.
582: In vivo nanoparticle-mediated therapeutic nucleic acid delivery for CF treatmentPiotrowski-Daspit A, Bracaglia L, Barone C, Nguyen R, Glazer P, Egan M, Saltzman W. 582: In vivo nanoparticle-mediated therapeutic nucleic acid delivery for CF treatment. Journal Of Cystic Fibrosis 2021, 20: s277. DOI: 10.1016/s1569-1993(21)02005-1.
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.
398: Host and pathogens contribute to decreased host defense protein SPLUNC1 in cystic fibrosisZielonka J, Khanal S, Webster M, Shiner N, Carpio A, Tarran R, Sharma L, DelaCruz C, Egan M, Britto C. 398: Host and pathogens contribute to decreased host defense protein SPLUNC1 in cystic fibrosis. Journal Of Cystic Fibrosis 2021, 20: s188. DOI: 10.1016/s1569-1993(21)01822-1.
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.
Understanding the Host in the Management of Pneumonia. An Official American Thoracic Society Workshop ReportDela Cruz CS, Evans SE, Restrepo MI, Dean N, Torres A, Amara-Elori I, Awasthi S, Caler E, Cao B, Chalmers JD, Chastre J, Cohen TS, Cohen AH, Crothers K, Di YP, Egan ME, Feldman C, Gautam S, Halstead ES, Herold S, Jones BE, Luna C, Niederman MS, Mendez R, Menendez R, Mizgerd JP, Nusrat R, Ramirez J, Shindo Y, Waterer G, Yeligar SM, Wunderink RG. Understanding the Host in the Management of Pneumonia. An Official American Thoracic Society Workshop Report. Annals Of The American Thoracic Society 2021, 18: 1087-1097. PMID: 34242148, PMCID: PMC8328365, DOI: 10.1513/annalsats.202102-209st.
SPLUNC1: a novel marker of cystic fibrosis exacerbationsKhanal S, Webster M, Niu N, Zielonka J, Nunez M, Chupp G, Slade MD, Cohn L, Sauler M, Gomez JL, Tarran R, Sharma L, Dela Cruz CS, Egan M, Laguna T, Britto CJ. SPLUNC1: a novel marker of cystic fibrosis exacerbations. European Respiratory Journal 2021, 58: 2000507. PMID: 33958427, PMCID: PMC8571118, DOI: 10.1183/13993003.00507-2020.
Nanoparticles for delivery of agents to fetal lungsUllrich SJ, Freedman-Weiss M, Ahle S, Mandl HK, Piotrowski-Daspit AS, Roberts K, Yung N, Maassel N, Bauer-Pisani T, Ricciardi AS, Egan ME, Glazer PM, Saltzman WM, Stitelman DH. Nanoparticles for delivery of agents to fetal lungs. Acta Biomaterialia 2021, 123: 346-353. PMID: 33484911, PMCID: PMC7962939, DOI: 10.1016/j.actbio.2021.01.024.
Emerging technologies for cystic fibrosis transmembrane conductance regulator restoration in all people with CFEgan 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.
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.
Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update.Egan ME. Cystic fibrosis transmembrane conductance receptor modulator therapy in cystic fibrosis, an update. Current Opinion In Pediatrics 2020, 32: 384-388. PMID: 32374578, DOI: 10.1097/mop.0000000000000892.
Global chemical effects of the microbiome include new bile-acid conjugationsQuinn RA, Melnik AV, Vrbanac A, Fu T, Patras KA, Christy MP, Bodai Z, Belda-Ferre P, Tripathi A, Chung LK, Downes M, Welch RD, Quinn M, Humphrey G, Panitchpakdi M, Weldon KC, Aksenov A, da Silva R, Avila-Pacheco J, Clish C, Bae S, Mallick H, Franzosa EA, Lloyd-Price J, Bussell R, Thron T, Nelson AT, Wang M, Leszczynski E, Vargas F, Gauglitz JM, Meehan MJ, Gentry E, Arthur TD, Komor AC, Poulsen O, Boland BS, Chang JT, Sandborn WJ, Lim M, Garg N, Lumeng JC, Xavier RJ, Kazmierczak BI, Jain R, Egan M, Rhee KE, Ferguson D, Raffatellu M, Vlamakis H, Haddad GG, Siegel D, Huttenhower C, Mazmanian SK, Evans RM, Nizet V, Knight R, Dorrestein PC. Global chemical effects of the microbiome include new bile-acid conjugations. Nature 2020, 579: 123-129. PMID: 32103176, PMCID: PMC7252668, DOI: 10.1038/s41586-020-2047-9.
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.
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.
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.
Sustainable Agriculture and Shortness of BreathSendon C, Rebaza A, Egan M. Sustainable Agriculture and Shortness of Breath. 2019, a4975-a4975. DOI: 10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a4975.
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.
Effects of Lumacaftor/Ivacaftor in a Pediatric Cohort Homozygous for F508del-CFTREgan ME. Effects of Lumacaftor/Ivacaftor in a Pediatric Cohort Homozygous for F508del-CFTR. American Journal Of Respiratory And Critical Care Medicine 2017, 195: 849-850. PMID: 28362199, DOI: 10.1164/rccm.201611-2290ed.
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.
Genetics of Cystic Fibrosis Clinical ImplicationsEgan ME. Genetics of Cystic Fibrosis Clinical Implications. Clinics In Chest Medicine 2015, 37: 9-16. PMID: 26857764, DOI: 10.1016/j.ccm.2015.11.002.
Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epitheliumMcNeer NA, Anandalingam K, Fields RJ, Caputo C, Kopic S, Gupta A, Quijano E, Polikoff L, Kong Y, Bahal R, Geibel JP, Glazer PM, Saltzman WM, Egan ME. Nanoparticles that deliver triplex-forming peptide nucleic acid molecules correct F508del CFTR in airway epithelium. Nature Communications 2015, 6: 6952. PMID: 25914116, PMCID: PMC4480796, DOI: 10.1038/ncomms7952.
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.
Association between serum 25‐hydroxyvitamin D level and pulmonary exacerbations in cystic fibrosisVanstone MB, Egan ME, Zhang JH, Carpenter TO. Association between serum 25‐hydroxyvitamin D level and pulmonary exacerbations in cystic fibrosis. Pediatric Pulmonology 2015, 50: 441-446. PMID: 25657016, DOI: 10.1002/ppul.23161.
Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trialConrad C, Lymp J, Thompson V, Dunn C, Davies Z, Chatfield B, Nichols D, Clancy J, Vender R, Egan M, Quittell L, Michelson P, Antony V, Spahr J, Rubenstein R, Moss R, Herzenberg L, Goss C, Tirouvanziam R. Long-term treatment with oral N-acetylcysteine: Affects lung function but not sputum inflammation in cystic fibrosis subjects. A phase II randomized placebo-controlled trial. Journal Of Cystic Fibrosis 2014, 14: 219-227. PMID: 25228446, DOI: 10.1016/j.jcf.2014.08.008.
Modified Poly(lactic‐co‐glycolic Acid) Nanoparticles for Enhanced Cellular Uptake and Gene Editing in the LungFields RJ, Quijano E, McNeer NA, Caputo C, Bahal R, Anandalingam K, Egan ME, Glazer PM, Saltzman WM. Modified Poly(lactic‐co‐glycolic Acid) Nanoparticles for Enhanced Cellular Uptake and Gene Editing in the Lung. Advanced Healthcare Materials 2014, 4: 361-366. PMID: 25156908, PMCID: PMC4339402, DOI: 10.1002/adhm.201400355.
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.
Baby bottle steam sterilizers disinfect home nebulizers inoculated with bacterial respiratory pathogensTowle D, Callan DA, Farrel PA, Egan ME, Murray TS. Baby bottle steam sterilizers disinfect home nebulizers inoculated with bacterial respiratory pathogens. Journal Of Cystic Fibrosis 2012, 12: 512-516. PMID: 23267773, DOI: 10.1016/j.jcf.2012.11.013.
Surface modified poly(β amino ester)-containing nanoparticles for plasmid DNA deliveryFields RJ, Cheng CJ, Quijano E, Weller C, Kristofik N, Duong N, Hoimes C, Egan ME, Saltzman WM. Surface modified poly(β amino ester)-containing nanoparticles for plasmid DNA delivery. Journal Of Controlled Release 2012, 164: 41-48. PMID: 23041278, PMCID: PMC3625648, DOI: 10.1016/j.jconrel.2012.09.020.
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.
Calcium-Modulated Chloride Pathways Contribute to Chloride Flux in Murine Cystic Fibrosis-Affected MacrophagesShenoy A, Kopic S, Murek M, Caputo C, Geibel JP, Egan ME. Calcium-Modulated Chloride Pathways Contribute to Chloride Flux in Murine Cystic Fibrosis-Affected Macrophages. Pediatric Research 2011, 70: 447-452. PMID: 21796019, PMCID: PMC3189336, DOI: 10.1203/pdr.0b013e31822f2448.
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.
Cystic Fibrosis Transmembrane Conductance Regulator Protein And Calcium Activated Chloride Channels Mediate Chloride Efflux In Murine MacrophagesShenoy A, Kopic S, Murek M, Caputo C, Geibel J, Egan M. Cystic Fibrosis Transmembrane Conductance Regulator Protein And Calcium Activated Chloride Channels Mediate Chloride Efflux In Murine Macrophages. 2010, a6575-a6575. DOI: 10.1164/ajrccm-conference.2010.181.1_meetingabstracts.a6575.
Partial Correction of Cystic Fibrosis Defects with PLGA Nanoparticles Encapsulating CurcuminCartiera MS, Ferreira EC, Caputo C, Egan ME, Caplan MJ, Saltzman WM. Partial Correction of Cystic Fibrosis Defects with PLGA Nanoparticles Encapsulating Curcumin. Molecular Pharmaceutics 2009, 7: 86-93. PMID: 19886674, PMCID: PMC2815009, DOI: 10.1021/mp900138a.
How useful are cystic fibrosis mouse models?Egan M. How useful are cystic fibrosis mouse models? Drug Discovery Today Disease Models 2009, 6: 35-41. DOI: 10.1016/j.ddmod.2009.03.009.
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.
Pseudomonas aeruginosa chronic colonization in cystic fibrosis patientsMurray TS, Egan M, Kazmierczak BI. Pseudomonas aeruginosa chronic colonization in cystic fibrosis patients. Current Opinion In Pediatrics 2007, 19: 83-88. PMID: 17224667, DOI: 10.1097/mop.0b013e3280123a5d.
ΔF508 Mutation Results in Impaired Gastric Acid Secretion*Sidani SM, Kirchhoff P, Socrates T, Stelter L, Ferreira E, Caputo C, Roberts KE, Bell RL, Egan ME, Geibel JP. ΔF508 Mutation Results in Impaired Gastric Acid Secretion*. Journal Of Biological Chemistry 2006, 282: 6068-6074. PMID: 17178714, DOI: 10.1074/jbc.m608427200.
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.
Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis DefectsEgan ME, Pearson M, Weiner SA, Rajendran V, Rubin D, Glöckner-Pagel J, Canny S, Du K, Lukacs GL, Caplan MJ. Curcumin, a Major Constituent of Turmeric, Corrects Cystic Fibrosis Defects. Science 2004, 304: 600-602. PMID: 15105504, DOI: 10.1126/science.1093941.
CFTR plays a major role in acid secretion in gastric glandsStelter L, Kirchhoff P, Boucher R, Grubb B, Wagner C, Egan M, Geibel J. CFTR plays a major role in acid secretion in gastric glands. Gastroenterology 2003, 124: a443. DOI: 10.1016/s0016-5085(03)82240-0.
Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cellsEgan ME, Glöckner-Pagel J, Ambrose C, Cahill PA, Pappoe L, Balamuth N, Cho E, Canny S, Wagner CA, Geibel J, Caplan MJ. Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells. Nature Medicine 2002, 8: 485-492. PMID: 11984593, DOI: 10.1038/nm0502-485.
Effects of the Serine/Threonine Kinase SGK1 on the Epithelial Na+ Channel (ENaC) and CFTR: Implications for Cystic FibrosisWagner 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.
Identification of the Cystic Fibrosis Transmembrane Conductance Regulator Domains That Are Important for Interactions with ROMK2*Cahill P, Nason M, Ambrose C, Yao T, Thomas P, Egan M. Identification of the Cystic Fibrosis Transmembrane Conductance Regulator Domains That Are Important for Interactions with ROMK2*. Journal Of Biological Chemistry 2000, 275: 16697-16701. PMID: 10748197, DOI: 10.1074/jbc.m910205199.
Continuous detection of extracellular ATP on living cells by using atomic force microscopySchneider S, Egan M, Jena B, Guggino W, Oberleithner H, Geibel J. Continuous detection of extracellular ATP on living cells by using atomic force microscopy. Proceedings Of The National Academy Of Sciences Of The United States Of America 1999, 96: 12180-12185. PMID: 10518596, PMCID: PMC18432, DOI: 10.1073/pnas.96.21.12180.
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.
CFTR is functionally active in GnRH-expressing GT1–7 hypothalamic neuronsWeyler 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.
A Novel ABC Transporter of the Sulfonylurea Receptor Family Expressed Specifically in the Drosophila Embryonic Heart and Tracheal SystemAlikasifoglu A, Nasonkin I, Cahill P, Ambrose C, Egan M, Thomas P. A Novel ABC Transporter of the Sulfonylurea Receptor Family Expressed Specifically in the Drosophila Embryonic Heart and Tracheal System. Pediatric Research 1999, 45: 84-84. DOI: 10.1203/00006450-199904020-00499.
CFTR Is a Conductance Regulator as well as a Chloride ChannelSCHWIEBERT E, BENOS D, EGAN M, STUTTS M, GUGGINO W. CFTR Is a Conductance Regulator as well as a Chloride Channel. Physiological Reviews 1999, 79: s145-s166. PMID: 9922379, DOI: 10.1152/physrev.1999.79.1.s145.
Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulatorSchwiebert E, Morales M, Devidas S, Egan M, Guggino W. Chloride channel and chloride conductance regulator domains of CFTR, the cystic fibrosis transmembrane conductance regulator. Proceedings Of The National Academy Of Sciences Of The United States Of America 1998, 95: 2674-2679. PMID: 9482946, PMCID: PMC19458, DOI: 10.1073/pnas.95.5.2674.
[49] Assays of dynamics, mechanisms, and regulation of ATP transport and release: Implications for study of ABC transporter functionSchwiebert E, Egan M, Guggino W. [49] Assays of dynamics, mechanisms, and regulation of ATP transport and release: Implications for study of ABC transporter function. 1998, 292: 664-675. PMID: 9711590, DOI: 10.1016/s0076-6879(98)92051-1.
In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR.Rubenstein R, Egan M, Zeitlin P. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. Journal Of Clinical Investigation 1997, 100: 2457-2465. PMID: 9366560, PMCID: PMC508446, DOI: 10.1172/jci119788.
A functional CFTR-NBF1 is required for ROMK2-CFTR interactionMcNicholas C, Nason M, Guggino W, Schwiebert E, Hebert S, Giebisch G, Egan M. A functional CFTR-NBF1 is required for ROMK2-CFTR interaction. American Journal Of Physiology 1997, 273: f843-f848. PMID: 9374850, DOI: 10.1152/ajprenal.1997.273.5.f843.
Recombinant adeno-associated virus (AAV-CFTR) vectors do not integrate in a site-specific fashion in an immortalized epithelial cell line.Kearns W, Afione S, Fulmer S, Pang M, Erikson D, Egan M, Landrum M, Flotte T, Cutting G. Recombinant adeno-associated virus (AAV-CFTR) vectors do not integrate in a site-specific fashion in an immortalized epithelial cell line. Gene Therapy 1996, 3: 748-55. PMID: 8875221.
Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator.McNicholas C, Guggino W, Schwiebert E, Hebert S, Giebisch G, Egan M. Sensitivity of a renal K+ channel (ROMK2) to the inhibitory sulfonylurea compound glibenclamide is enhanced by coexpression with the ATP-binding cassette transporter cystic fibrosis transmembrane regulator. Proceedings Of The National Academy Of Sciences Of The United States Of America 1996, 93: 8083-8088. PMID: 8755607, PMCID: PMC38879, DOI: 10.1073/pnas.93.15.8083.
CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATPSchwiebert E, Egan M, Hwang T, Fulmer S, Allen S, Cutting G, Guggino W. CFTR regulates outwardly rectifying chloride channels through an autocrine mechanism involving ATP. Cell 1995, 81: 1063-1073. PMID: 7541313, DOI: 10.1016/s0092-8674(05)80011-x.
Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cellsEgan M, Schwiebert E, Guggino W. Differential expression of ORCC and CFTR induced by low temperature in CF airway epithelial cells. American Journal Of Physiology 1995, 268: c243-c251. PMID: 7530908, DOI: 10.1152/ajpcell.1995.268.1.c243.
Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus OocytesCarroll T, Mclntosh I, Egan M, Zeitlin P, Cutting G, Guggino W. Transmembrane Mutations Alter the Channel Characteristics of the Cystic Fibrosis Transmembrane Conductance Regulator Expressed in Xenopus Oocytes. Cellular Physiology And Biochemistry 1994, 4: 10-18. DOI: 10.1159/000154705.
Modulation of Ion Transport in Cultured Rabbit Tracheal Epithelium by Lipoxygenase MetabolitesEgan M, Wagner M, Zeitlin P, Guggino W. Modulation of Ion Transport in Cultured Rabbit Tracheal Epithelium by Lipoxygenase Metabolites. American Journal Of Respiratory Cell And Molecular Biology 1992, 7: 500-506. PMID: 1419026, DOI: 10.1165/ajrcmb/7.5.500.
Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTREgan M, Flotte T, Afione S, Solow R, Zeitlin P, Carter B, Guggino W. Defective regulation of outwardly rectifying Cl− channels by protein kinase A corrected by insertion of CFTR. Nature 1992, 358: 581-584. PMID: 1380129, DOI: 10.1038/358581a0.
Kinetic Interaction of Fatty Acids with Outwardly Rectifying Chloride ChannelsHwang T, Egan M, Guggino W. Kinetic Interaction of Fatty Acids with Outwardly Rectifying Chloride Channels. Cellular Physiology And Biochemistry 1992, 2: 308-315. DOI: 10.1159/000154653.

Clinical Trials

Conditions	Study Title
Diseases of the Endocrine System; Genetics - Adult; Genetics - Pediatric	VX-121 Combination Therapy in Subjects With Cystic Fibrosis VX20-121-103
Hepatitis; HIV/AIDS; Immune System; Infectious Diseases	Screening In Anticipation of Future Research

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Marie Egan, MD

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