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Marsico Lung Institute In Collaboration with the Department of Genetics

Publications

  1. Belfield-Simpson L., Martin J.R., McPeek M.K., Livraghi-Butrico A., Dang H., Kim Y.H., Gilmour M.I., Doerschuk C.M.. Combustion products of burn pit constituents induce more changes in asthmatic than non-asthmatic murine lungs. Part Fibre Toxicol. 2025 Jul 29;22(1):21.
  2. Edwards D.A., Edwards A., Li D., Wang L., Chung K.F., Bhatta D., Bilstein A., Hanes J., Endirisinghe I., Freeman B.B., Gutay M., Livraghi-Butrico A., Button B. Global warming risks dehydrating and inflaming human airways. Commun Earth Environ. 2025;6(1):193.
  3. Heinzelmann K., Fysikopoulos A., Jaquin T.J., Peper-Gabriel J.K., Hansbauer E.M., Grüner S., Prassler J., Wurzenberger C., Kennedy J.G.C., Snead J.Y., Wrennall J.A., Heinig K., Wurzenberger C., Bel Aiba R.S., Tarran R., Livraghi-Butrico A., Fitzgerald M.F., Anderson G.P., Rothe C., Matschiner G., Olwill S.A., Hagner M. Pulmonary-delivered Anticalin Jagged-1 antagonists reduce experimental airway mucus hyperproduction and obstruction. Am J Physiol Lung Cell Mol Physiol. 2025 Jan 1;328(1):L75-L92. doi: 10.1152/ajplung.00059.2024. Epub 2024 Nov 5.
  4. Pearce CM, Shaw TD, Podell B, Jackson M, Henao-Tamayo M, Obregon-Henao A, Lam H, Alshiraihi IM, O’Neal W, Livraghi-Butrico A, Hickey AJ, Meibohm B, Gonzalez- Juarrero M. Mycobacterium abscessus pulmonary infection and associated respiratory function in cystic fibrosis-like βENaC mice. Front Tuberc. 2024;2:1473341.
  5. Nakano K., Whitehead G.S., Lyons-Cohen M.R., Grimm S.A., Wilkinson C.L., Izumi G., Livraghi-Butrico A., Cook D.N., Nakano H. Chemokine CCL19 promotes type 2 T-cell differentiation and allergic airway inflammation. J Allergy Clin Immunol. 2024 Feb;153(2):487-502.e9. doi: 10.1016/j.jaci.2023.10.024 Epub 2023 Nov 11. PMCID: PMC10922373 (available on 2025-02-01)
  6. Donoghue L.J., Markovetz M.R., Morrison C.B., Chen G., McFadden K.M., Sadritabrizi T.,Gutay M.I., Kato T., Rogers T.D., Snead J.Y., Livraghi-Butrico A, Button B., Ehre C.,Grubb B.R., Hill D.B., Kelada S.N.P. BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance. Am J Physiol Lung Cell Mol Physiol. 2023 Dec 1;325(6):L765-L775..
  7. Yin W., Golliher H.L., Ferguson A.J., Kimbell J.S., Livraghi-Butrico A., Rogers T.D., Grubb B.R., Kimple A.J., Ostrowski L.E. Mucolytic treatment of chronic rhinosinusitis in a murine model of primary ciliary dyskinesia. Front Mol Biosci. 2023 Jul 24;10:1221796. eCollection 2023.
  8. Biering S.B., Sarnik S.A., Wang E., Zengel J.R., Leist S.R., Schäfer A., Sathyan V., Hawkins P., Okuda K., Tau C., Jangid A.R., Duffy C.V., Wei J., Gilmore R.C., Alfajaro M.M., Strine M.S., Nguyenla X., Van Dis E., Catamura C., Yamashiro L.H., Belk J.A., Begeman A., Stark J.C., Shon D.J., Fox D.M., Ezzatpour S., Huang E., Olegario N., Rustagi A., Volmer A.S., Livraghi-Butrico A., Wehri E., Behringer R.R., Cheon D.J., Schaletzky J., Aguilar H.C., Puschnik A.S., Button B., Pinsky B.A., Blish C.A., Baric R.S., O’Neal W.K., Bertozzi C.R., Wilen C.B., Boucher R.C., Carette J.E., Stanley S.A., Harris E., Konermann S., Hsu P.D. Genome-wide bidirectional CRISPR screens identify mucins as host factors modulating SARS-CoV-2 infection. Nat Genet. 2022 Aug;54(8):1078-1089. Epub 2022 Jul 25.
  9. Kim N., Rodriguez J., Livraghi-Butrico A., Shenoy S., Mazur M., Birket S.E., Rowe S.M., Boucher R.C., Hanes J., Suk J.S. Inhaled gene therapy of preclinical muco-obstructive lung diseases by nanoparticles capable of breaching the airway mucus barrier”. 2022 Aug;77(8):812-820.
  10. Rogers T.D., Button B., Kelada S.N.P., Ostrowski L.E., Livraghi-Butrico A., Gutay M.I., Esther, C.R.Jr, Grubb, B.R. Regional Differences in Mucociliary Clearance in the Upper and Lower Airways. Frontiers in Physiology. 2022;13 09 March 9; 13:842592.
  11. Xu J.*, Livraghi-Butrico A.*, Hou X.*, Rajagopalan C.*, Zhang J., Song J., Jiang H., Wei H-G., Wang H., Bouhamdan M., Ruan ,Yang D., Qiu Y., Youming X., Barrett R., McClellan S.A., Mou H., Wu Q., Chen X., Rogers T.D., Wilkinson K.J., Gilmore R.C., Esther C.R. Jr., Zaman K., Liang X., Sobolic M., Hazlett L., Zhang K., Frizzell R,A., Gentzsch M., O’Neal W.K., Grubb B.R., Chen Y.E.#, Boucher R.C. #, Sun F. # Phenotypes of CF rabbits generated by CRISPR/Cas9-mediated disruption of the CFTR gene. JCI Insight. 2021 Jan 11;6(1):e139813. *Co-first authors, #Co-senior authors.
  12. Sigmon J.S., Blanchard M.W., Baric R.S., Bell T.A., Brennan J., Brockmann G.A., Burks A.W., Calabrese J..M, Caron K.M., Cheney R.E., Ciavatta D., Conlon F., Darr D.B., Faber J., Franklin C., Gershon T.R., Gralinski L., Gu B., Gaines C.H., Hagan R.S., Heimsath E.G., Heise M.T., Hock P., Ideraabdullah F., Jennette J.C., Kafri T., Kashfeen A., Kulis M., Kumar V., Linnertz C., Livraghi-Butrico A., Lloyd K.C.K, Lutz C., Lynch R.M., Magnuson T., Matsushima G.K., McMullan R., Miller D.R., Mohlke K.L., Moy S.S., Murphy C., Najarian M., O’Brien L., Palmer A.A., Philpot B.D., Randell S.H., Reinholdt L., Ren Y., Rockwood S., Rogala A.R., Saraswatula A., Sassetti C.M., Schisler J.C., Schoenrock S.A., Shaw G.D., Shorter J.R., Smith C.M., St Pierre C.L., Tarantino L.M., Threadgill D.W., Valdar W., Vilen B.J., Wardwell K., Whitmire J.K., Williams L., Zylka M.J., Ferris M.T., McMillan L., Pardo- Manuel de Villena F. Content and Performance of the MiniMUGA Genotyping Array, a New Tool To Improve Rigor and Reproducibility in Mouse Research. 2020 Dec;216(4):905-930.
  13. Chen G., Sun L., Kato T., Okuda K., Martino M.B., Abzhanova A., Lin J.M., Gilmore R.C., Batson B.D., O’Neal Y.K., Volmer A.S., Dang H., Deng Y., Randell S.H., Button B., Livraghi- Butrico A., Kesimer M., Ribeiro C.M., O’Neal W.K., Boucher R.C. IL-1β dominates the pro-mucin secretory cytokine profile in cystic fibrosis. J Clin Invest. 2019 Oct 1;129(10):4433-4450.
  14. Brinks V., Lipinska K., de Jager M., Beumer W., Button B., Livraghi-Butrico A., Henig N., Matthee B. The Cystic Fibrosis-Like Airway Surface Layer is not a Significant Barrier for Delivery of Eluforsen to Airway Epithelial Cells. J Aerosol Med Pulm Drug Deliv. 2019 Oct;32(5):303-316.
  15. Yin W., Livraghi-Butrico A., Sears P.R., Rogers T.D., Burns K.A., Grubb B.R., Ostrowski L.E. Mice with a Deletion of Rsph1 Exhibit a Low Level of Mucociliary Clearance and Develop a PCD Phenotype. Am J Respir Cell Mol Biol. 2019 Sep;61(3):312-321.
  16. Chen G., Ribeiro C.M.P., Sun L., Okuda K., Kato T., Gilmore R.C., Martino M.B., Dang H., Abzhanova A., Lin J.M., Hull-Ryde E.A., Volmer A.S., Randell S.H., Livraghi-Butrico A., Deng Y., Scherer P.E., Stripp B.R., O’Neal W.K., Boucher R.C. XBP1S Regulates MUC5B in a Promoter Variant-Dependent Pathway in IPF Airway Epithelia. Am J Respir Crit Care Med. 2019 Jul 15;200(2):220-234.
  17. Ehre C., Rushton Z.L., Wang B., Hothem L.N., Morrison C.B., Fontana N.C., Markovetz M.R., Delion M.F., Kato T., Villalon D., Thelin W.R., Esther C.R. Jr., Hill D.B., Grubb B.R., Livraghi-Butrico A., Donaldson S.H., Boucher R.C. An Improved Inhaled Mucolytic to Treat Airway Muco-Obstructive Diseases. Am J Respir Crit Care Med. 2019 Jan 15;199(2):171-180.
  18. Rogers T.D., Ostrowski L.E., Livraghi-Butrico A. Button B., Grubb B.R. Mucociliary clearance in mice measured by tracking trans-tracheal fluorescence of nasally aerosolized beads. Sci Rep. 2018 Oct 3;8(1):14744.
  19. Chen G., Volmer A.S., Wilkinson K.J., Deng Y., Jones L.C., Yu D., Bustamante-Marin X.M., Burns K.A., Grubb B.R., O’Neal W.K., Livraghi-Butrico A., Boucher R.C. Role of Spdef in the regulation of Muc5b expression in the airways of naïve and muco-obstructed mice. Am J Respir Cell Mol Biol. 2018 Sep;59(3):383-396.
  20. Ghosh A., Coakley R.C., Mascenik T., Rowell T.R., Davis E.S., Rogers K., Webster M.J., Dang H., Herring L.E., Sassano M.F., Livraghi-Butrico A., Van Buren S.K., Graves L.M., Herman M.A., Randell S.H., Alexis N.E., Tarran R. Chronic E-cigarette Exposure Alters the Human Bronchial Epithelial Proteome. Am J Respir Crit Care Med. 2018 Jul 1;198(1):67-76.
  21. Saini Y., Lewis B.W., Yu D., Dang H., Livraghi-Butrico A., Del Piero F., O’Neal W.K., Boucher R.C. Effect of LysM+ macrophage depletion on lung pathology in mice with chronic bronchitis. Physiol Rep. 2018 Apr;6(8): e13677.
  22. Duncan G.A., Kim N., Colon-Cortes Y., Rodriguez J., Mazur M., Birket S.E., Rowe S.M., West N.E., Livraghi-Butrico A., Boucher R.C., Hanes J., Aslanidi G., Suk J.S. An Adeno-Associated Viral Vector Capable of Penetrating the Mucus Barrier to Inhaled Gene Therapy. Mol Ther Methods Clin Dev. 2018 Mar 22; 9:296-304.
  23. Livraghi-Butrico A., Wilkinson K.J., Volmer A.S., Gilmore R.C., Rogers, T.D., Caldwell, R.A, Burns, K.A., Esther, C.R. Jr., Mall, M.A., Boucher, R.C., O’Neal, W.K., Grubb, B.R. Lung disease phenotypes caused by over-expression of combinations of alpha, beta, and gamma subunits of the epithelial sodium channel in mouse airways. Am J Physiol Lung Cell Mol Physiol. 2018 Feb 1;314(2): L318-L331.
  24. Donoghue L.J.*, Livraghi-Butrico A.*, McFadden K.M., Thomas J.M., Chen G., Grubb B.R., O’Neal W.K., Boucher R.C., Kelada S.N.P. Identification of trans Protein QTL for Secreted Airway Mucins in Mice and a Causal Role for Bpifb1. 2017 Oct;207(2):801-812. *Co-first authors
  25. Livraghi-Butrico A., Grubb B.R., Wilkinson K.J., Volmer A.S., Burns K.A., Evans C.M., O’Neal W.K., Boucher R.C. Contribution of mucus concentration and secreted mucins Muc5ac and Muc5b to the pathogenesis of muco-obstructive lung disease. Mucosal Immunol. 2017 Mar;10(2):395-407.
  26. Sesma J.I., Weitzer C.D., Livraghi-Butrico A., Dang H., Donaldson S., Alexis N.E., Jacobson K.A., Harden T.K., Lazarowski E.R. UDP-glucose promotes neutrophil recruitment in the lung. Purinergic Signal. 2016 Dec;12(4):627-635.
  27. Grubb B.R, Livraghi-Butrico A., Rogers T., Yin W-N, Ostrowski L.E. Reduced mucociliary clearance in old mice is associated with a decrease in Muc5B mucin. Am J Physiol Lung Cell Mol Physiol. 2016 May 1;310(9):L860-7.
  28. Saini Y., Wilkinson, K.J., Terrell K.A., Burns K.A., Livraghi-Butrico A., Doerschuk C.M., O’Neal W.K., Boucher R.C. Neonatal Pulmonary Macrophage Depletion Coupled to Defective Mucus Clearance Increases Susceptibility to Pneumonia and Alters Pulmonary Immune Responses. Am J Respir Cell Mol Biol. 2016 Feb;54(2):210-21.
  29. Saini Y., Dang H., Livraghi-Butrico A., Kelly E.J., Jones L.C., O’Neal W.K., Boucher R.C. Gene expression in whole lung and pulmonary macrophages reflects the dynamic pathology associated with airway surface dehydration. BMC Genomics. 2014 Sep 10; 15:726.
  30. Roy M.G.*, Livraghi-Butrico A.*, Fletcher A.A.*, McElwee M.M., Evans S.E., Boerner R.M., Alexander S.N., Bellinghausen L.K., Song A.S., Petrova Y.M., Tuvim M.J., Adachi R., Romo I., Bordt A.S., Bowden M.G., Sisson J.H., Woodruff, P.G., Thornton, D.J., Rousseau K., De la Garza M.M., Moghaddam S.J., Karmouty-Quintana H., Blackburn, M.R., Drouin S.M., Davis C.W., Terrell K.A., Grubb B.R., O’Neal W.K., Flores S.C., Cota-Gomez, A., Lozupone C.A., Donnelly J.A., Watson, A.M., Hennessy C.E., Keith R.C.,Yang I.V., Barthel, L., Peter M. Henson P.M., Janssen W.J., Schwartz, D.A., Boucher, R.C., Dickey B.F., Evans C.M. Muc5b Is Required for Airway Defense. 2014 Jan 16;505(7483):412-6.
  31. Livraghi-Butrico A., Kelly E.J., Wilkinson K.J., Rogers T.D., Gilmore R.C, Harkema J.R., Randell S.H., Boucher R.C., O’Neal W.K., Grubb B.R. Loss of Cftr function exacerbates the phenotype of Na+ hyperabsorption in murine airways. Am J Physiol Lung Cell Mol Physiol. 2013 Apr 1;304(7):L469-80.
  32. Livraghi-Butrico A., Kelly E.J., Klem E.R., Dang H., Wolfgang M.C., Boucher R.C., Randell S.H., O’Neal W.K. Mucus clearance, MyD88-dependent and MyD88-independent immunity modulate lung susceptibility to spontaneous bacterial infection and inflammation. Mucosal Immunol. 2012 Jul;5(4):397-408.
  33. Livraghi-Butrico, A., Grubb B.R., Kelly E.J., Wilkinson K.J., Yang H., Geiser M., Randell S.H., Boucher R.C., O’Neal W.K. Genetically Determined Heterogeneity of Lung Disease in a Mouse Model of Airway Mucus Obstruction. Physiol Genomics. 2012 Apr 15;44(8):470-84.
  34. Mall M.A., Button B., Johannesson B., Zhou Z., Livraghi A., Caldwell R.A., Schubert S.C., Schultz C., O’Neal W.K., Pradervand S., Hummler E., Rossier B.C., Grubb B.R., Boucher R.C. Airway surface liquid volume regulation determines different airway phenotypes in Liddle compared with betaENaC-overexpressing mice. J Biol Chem. 2010 Aug 27;285(35):26945-55.
  35. Livraghi A., Grubb B.R., Hudson E.J., Wilkinson K.J., Sheehan J.K., Mall M.A., O’Neal W.K., Boucher R.C., Randell S.H. Airway and lung pathology due to mucosal surface dehydration in β-epithelial Na+ channel-overexpressing mice: role of TNF-α and IL-4Rα signaling, influence of neonatal development, and limited efficacy of glucocorticoid treatment. J Immunol. 2009 Apr 1;182(7):4357-67.
  36. Rowe S.M., Jackson P.L., Liu G., Hardison M., Livraghi A., Solomon G.M., McQuaid D.B., Noerager B.D., Gaggar A., Clancy J.P., O’Neal W.K., Sorscher E.J., Abraham E., Blalock J.E. Potential role of high-mobility group box 1 in cystic fibrosis airway disease. Am J Respir Crit Care Med. 2008 Oct 15;178(8):822-31.
  37. Mall M.A., Harkema J.R., Trojanek J.B., Treis D., Livraghi A., Schubert S., Zhou, Z., Kreda S.M., Tilley S.L., Hudson E.J., O’Neal W.K., Boucher R.C. Development of chronic bronchitis and emphysema in beta-epithelial Na+ channel-overexpressing mice. Am J Respir Crit Care Med. 2008 Apr 1;177(7):730-42.