Publications (2008-present)

Precision Genomic Medicine in Cystic Fibrosis. 
Chang EH, Zabner J.
Clin Transl Sci. 2015 Jun 15. doi: 10.1111/cts.12292. [Epub ahead of print]
PMID: 26073768

Characterization of fusion genes and the significantly expressed fusion isoforms in breast cancer by hybrid sequencing. 
Weirather JL, Afshar PT, Clark TA, Tseng E, Powers LS, Underwood JG, Zabner J, Korlach J, Wong WH, Au KF.
Nucleic Acids Res. 2015 Jun 3. pii: gkv562. [Epub ahead of print]
PMID: 26040699

Starting a lung transplant program: a roadmap for long-term excellence. 
Klesney-Tait J, Eberlein M, Geist L, Keech J, Zabner J, Gruber PJ, Iannettoni MD, Parekh K.
Chest. 2015 May;147(5):1435-43. doi: 10.1378/chest.14-2241.
PMID: 25940255

pH modulates the activity and synergism of the airway surface liquid antimicrobials β-defensin-3 and LL-37. 
Abou Alaiwa MH, Reznikov LR, Gansemer ND, Sheets KA, Horswill AR, Stoltz DA, Zabner J, Welsh MJ.
Proc Natl Acad Sci U S A. 2014 Dec 30;111(52):18703-8. doi: 10.1073/pnas.1422091112. Epub 2014 Dec 15.
PMID: 25512526

Aggregates of mutant CFTR fragments in airway epithelial cells of CF lungs: new pathologic observations. 
Du K, Karp PH, Ackerley C, Zabner J, Keshavjee S, Cutz E, Yeger H.
J Cyst Fibros. 2015 Mar;14(2):182-93. doi: 10.1016/j.jcf.2014.09.012. Epub 2014 Oct 28.
PMID: 25453871

Medical reversal of chronic sinusitis in a cystic fibrosis patient with ivacaftor. 
Chang EH, Tang XX, Shah VS, Launspach JL, Ernst SE, Hilkin B, Karp PH, Abou Alaiwa MH, Graham SM, Hornick DB, Welsh MJ, Stoltz DA, Zabner J.
Int Forum Allergy Rhinol. 2015 Feb;5(2):178-81. doi: 10.1002/alr.21440. Epub 2014 Oct 31.
PMID: 25363320

Iron oxide nanoparticles induce Pseudomonas aeruginosa growth, induce biofilm formation, and inhibit antimicrobial peptide function. 
Borcherding J, Baltrusaitis J, Chen H, Stebounova L, Wu CM, Rubasinghege G, Mudunkotuwa IA, Caraballo JC, Zabner J, Grassian VH, Comellas AP.
Environ Sci Nano. 2014 Apr;1(2):123-132.
PMID: 25221673

Impaired mucus detachment disrupts mucociliary transport in a piglet model of cystic fibrosis. 
Hoegger MJ, Fischer AJ, McMenimen JD, Ostedgaard LS, Tucker AJ, Awadalla MA, Moninger TO, Michalski AS, Hoffman EA, Zabner J, Stoltz DA, Welsh MJ.
Science. 2014 Aug 15;345(6198):818-22. doi: 10.1126/science.1255825.
PMID: 25124441

Mitochondrial-targeted antioxidant therapy decreases transforming growth factor-β-mediated collagen production in a murine asthma model. 
Jaffer OA, Carter AB, Sanders PN, Dibbern ME, Winters CJ, Murthy S, Ryan AJ, Rokita AG, Prasad AM, Zabner J, Kline JN, Grumbach IM, Anderson ME.
Am J Respir Cell Mol Biol. 2015 Jan;52(1):106-15. doi: 10.1165/rcmb.2013-0519OC.
PMID: 24988374

Neonates with cystic fibrosis have a reduced nasal liquid pH; a small pilot study. 
Abou Alaiwa MH, Beer AM, Pezzulo AA, Launspach JL, Horan RA, Stoltz DA, Starner TD, Welsh MJ, Zabner J.
J Cyst Fibros. 2014 Jul;13(4):373-7. doi: 10.1016/j.jcf.2013.12.006. Epub 2014 Jan 11.
PMID: 24418186

Role of PON in anoxia-reoxygenation injury: a Drosophila melanogaster transgenic model. 
Caraballo JC, Borcherding J, Rector M, Hornick E, Stoltz D, Zabner J, Comellas AP.
PLoS One. 2014 Jan 6;9(1):e84434. doi: 10.1371/journal.pone.0084434. eCollection 2014.
PMID: 24400090

Integrin α6β4 identifies human distal lung epithelial progenitor cells with potential as a cell-based therapy for cystic fibrosis lung disease. 
Li X, Rossen N, Sinn PL, Hornick AL, Steines BR, Karp PH, Ernst SE, Adam RJ, Moninger TO, Levasseur DN, Zabner J.
PLoS One. 2013 Dec 12;8(12):e83624. doi: 10.1371/journal.pone.0083624. eCollection 2013.
PMID: 24349537

CaMKII is essential for the proasthmatic effects of oxidation. 
Sanders PN, Koval OM, Jaffer OA, Prasad AM, Businga TR, Scott JA, Hayden PJ, Luczak ED, Dickey DD, Allamargot C, Olivier AK, Meyerholz DK, Robison AJ, Winder DG, Blackwell TS, Dworski R, Sammut D, Wagner BA, Buettner GR, Pope RM, Miller FJ Jr, Dibbern ME, Haitchi HM, Mohler PJ, Howarth PH, Zabner J, Kline JN, Grumbach IM, Anderson ME.
Sci Transl Med. 2013 Jul 24;5(195):195ra97. doi: 10.1126/scitranslmed.3006135.
PMID: 23884469

Abundant DNase I-sensitive bacterial DNA in healthy porcine lungs and its implications for the lung microbiome. 
Pezzulo AA, Kelly PH, Nassar BS, Rutland CJ, Gansemer ND, Dohrn CL, Costello AJ, Stoltz DA, Zabner J.
Appl Environ Microbiol. 2013 Oct;79(19):5936-41. doi: 10.1128/AEM.01752-13. Epub 2013 Jul 19.
PMID: 23872563

Protein composition of bronchoalveolar lavage fluid and airway surface liquid from newborn pigs. 
Bartlett JA, Albertolle ME, Wohlford-Lenane C, Pezzulo AA, Zabner J, Niles RK, Fisher SJ, McCray PB Jr, Williams KE.
Am J Physiol Lung Cell Mol Physiol. 2013 Aug 1;305(3):L256-66. doi: 10.1152/ajplung.00056.2013. Epub 2013 May 24.
PMID: 23709621

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs. 
Stoltz DA, Rokhlina T, Ernst SE, Pezzulo AA, Ostedgaard LS, Karp PH, Samuel MS, Reznikov LR, Rector MV, Gansemer ND, Bouzek DC, Alaiwa MH, Hoegger MJ, Ludwig PS, Taft PJ, Wallen TJ, Wohlford-Lenane C, McMenimen JD, Chen JH, Bogan KL, Adam RJ, Hornick EE, Nelson GA 4th, Hoffman EA, Chang EH, Zabner J, McCray PB Jr, Prather RS, Meyerholz DK, Welsh MJ.
J Clin Invest. 2013 Jun;123(6):2685-93. doi: 10.1172/JCI68867. Epub 2013 May 8.
PMID: 23676501

Adenoviral gene transfer corrects the ion transport defect in the sinus epithelia of a porcine CF model. 
Potash AE, Wallen TJ, Karp PH, Ernst S, Moninger TO, Gansemer ND, Stoltz DA, Zabner J, Chang EH.
Mol Ther. 2013 May;21(5):947-53. doi: 10.1038/mt.2013.49. Epub 2013 Mar 19.
PMID: 23511247

Coal fly ash impairs airway antimicrobial peptides and increases bacterial growth. 
Borcherding JA, Chen H, Caraballo JC, Baltrusaitis J, Pezzulo AA, Zabner J, Grassian VH, Comellas AP.
PLoS One. 2013;8(2):e57673. doi: 10.1371/journal.pone.0057673. Epub 2013 Feb 28.
PMID: 23469047

Transepithelial migration of neutrophils into the lung requires TREM-1. 
Klesney-Tait J, Keck K, Li X, Gilfillan S, Otero K, Baruah S, Meyerholz DK, Varga SM, Knudson CJ, Moninger TO, Moreland J, Zabner J, Colonna M.
J Clin Invest. 2013 Jan;123(1):138-49. doi: 10.1172/JCI64181. Epub 2012 Dec 17.
PMID: 23241959

Expression of human paraoxonase 1 decreases superoxide levels and alters bacterial colonization in the gut of Drosophila melanogaster. 
Pezzulo AA, Hornick EE, Rector MV, Estin M, Reisetter AC, Taft PJ, Butcher SC, Carter AB, Manak JR, Stoltz DA, Zabner J.
PLoS One. 2012;7(8):e43777. doi: 10.1371/journal.pone.0043777. Epub 2012 Aug 30.
PMID: 22952763

Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. 
Pezzulo AA, Tang XX, Hoegger MJ, Alaiwa MH, Ramachandran S, Moninger TO, Karp PH, Wohlford-Lenane CL, Haagsman HP, van Eijk M, Bánfi B, Horswill AR, Stoltz DA, McCray PB Jr, Welsh MJ, Zabner J.
Nature. 2012 Jul 4;487(7405):109-13. doi: 10.1038/nature11130.
PMID: 22763554

Sinus hypoplasia precedes sinus infection in a porcine model of cystic fibrosis. 
Chang EH, Pezzulo AA, Meyerholz DK, Potash AE, Wallen TJ, Reznikov LR, Sieren JC, Karp PH, Ernst S, Moninger TO, Gansemer ND, McCray PB Jr, Stoltz DA, Welsh MJ, Zabner J.
Laryngoscope. 2012 Sep;122(9):1898-905. doi: 10.1002/lary.23392. Epub 2012 Jun 18.
PMID: 22711071

CFTR is required for maximal transepithelial liquid transport in pig alveolar epithelia. 
Li X, Comellas AP, Karp PH, Ernst SE, Moninger TO, Gansemer ND, Taft PJ, Pezzulo AA, Rector MV, Rossen N, Stoltz DA, McCray PB Jr, Welsh MJ, Zabner J.
Am J Physiol Lung Cell Mol Physiol. 2012 Jul;303(2):L152-60. doi: 10.1152/ajplung.00116.2012. Epub 2012 May 25.
PMID: 22637155

Hoechst increases adeno-associated virus-mediated transgene expression in airway epithelia by inducing the cytomegalovirus promoter. 
Dickey DD, Excoffon KJ, Young KR, Parekh KR, Zabner J.
J Gene Med. 2012 Jun;14(6):366-73. doi: 10.1002/jgm.2632.
PMID: 22610695

Safety assessment of nebulized xylitol in beagle dogs. 
Reed MD, McCombie BE, Sivillo AE, Thorne PS, Welsh MJ, March TH, McDonald JD, Seilkop SK, Zabner J, Durairaj L.
Inhal Toxicol. 2012 May;24(6):365-72. doi: 10.3109/08958378.2012.673180.
PMID: 22564094

In Situ Quantification of Glucose Concentration in Airway Surface Liquid With Functionalized ZnO Nanorod-Coated Microelectrodes. 
Pezzulo AA, Asif MH, Willander M, Zabner J.
J Anal Bioanal Tech. 2011 Aug 12;S7(2). pii: 002.
PMID: 23227447

Requirements for ion and solute transport, and pH regulation during enamel maturation. 
Lacruz RS, Smith CE, Moffatt P, Chang EH, Bromage TG, Bringas P Jr, Nanci A, Baniwal SK, Zabner J, Welsh MJ, Kurtz I, Paine ML.
J Cell Physiol. 2012 Apr;227(4):1776-85. doi: 10.1002/jcp.22911.
PMID: 21732355

Enhanced sialic acid-dependent endocytosis explains the increased efficiency of infection of airway epithelia by a novel adeno-associated virus. 
Dickey DD, Excoffon KJ, Koerber JT, Bergen J, Steines B, Klesney-Tait J, Schaffer DV, Zabner J.
J Virol. 2011 Sep;85(17):9023-30. doi: 10.1128/JVI.05154-11. Epub 2011 Jun 22.
PMID: 21697483

Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance. 
Itani OA, Chen JH, Karp PH, Ernst S, Keshavjee S, Parekh K, Klesney-Tait J, Zabner J, Welsh MJ.
Proc Natl Acad Sci U S A. 2011 Jun 21;108(25):10260-5. doi: 10.1073/pnas.1106695108. Epub 2011 Jun 6.
PMID: 21646513

Enamel pathology resulting from loss of function in the cystic fibrosis transmembrane conductance regulator in a porcine animal model. 
Chang EH, Lacruz RS, Bromage TG, Bringas P Jr, Welsh MJ, Zabner J, Paine ML.
Cells Tissues Organs. 2011;194(2-4):249-54. doi: 10.1159/000324248. Epub 2011 Apr 28.
PMID: 21525720

The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. 
Ostedgaard LS, Meyerholz DK, Chen JH, Pezzulo AA, Karp PH, Rokhlina T, Ernst SE, Hanfland RA, Reznikov LR, Ludwig PS, Rogan MP, Davis GJ, Dohrn CL, Wohlford-Lenane C, Taft PJ, Rector MV, Hornick E, Nassar BS, Samuel M, Zhang Y, Richter SS, Uc A, Shilyansky J, Prather RS, McCray PB Jr, Zabner J, Welsh MJ, Stoltz DA.
Sci Transl Med. 2011 Mar 16;3(74):74ra24. doi: 10.1126/scitranslmed.3001868.
PMID: 21411740

Concentration of the antibacterial precursor thiocyanate in cystic fibrosis airway secretions. 
Lorentzen D, Durairaj L, Pezzulo AA, Nakano Y, Launspach J, Stoltz DA, Zamba G, McCray PB Jr, Zabner J, Welsh MJ, Nauseef WM, Bánfi B.
Free Radic Biol Med. 2011 May 1;50(9):1144-50. doi: 10.1016/j.freeradbiomed.2011.02.013. Epub 2011 Feb 18.
PMID: 21334431

Glucose depletion in the airway surface liquid is essential for sterility of the airways. 
Pezzulo AA, Gutiérrez J, Duschner KS, McConnell KS, Taft PJ, Ernst SE, Yahr TL, Rahmouni K, Klesney-Tait J, Stoltz DA, Zabner J.
PLoS One. 2011 Jan 20;6(1):e16166. doi: 10.1371/journal.pone.0016166.
PMID: 21311590

Do cell junction protein mutations cause an airway phenotype in mice or humans? 
Chang EH, Pezzulo AA, Zabner J.
Am J Respir Cell Mol Biol. 2011 Aug;45(2):202-20. doi: 10.1165/rcmb.2010-0498TR. Epub 2011 Feb 4. Review.
PMID: 21297078

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. 
Chen JH, Stoltz DA, Karp PH, Ernst SE, Pezzulo AA, Moninger TO, Rector MV, Reznikov LR, Launspach JL, Chaloner K, Zabner J, Welsh MJ.
Cell. 2010 Dec 10;143(6):911-23. doi: 10.1016/j.cell.2010.11.029.
PMID: 21145458

Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth. 
Rogan MP, Reznikov LR, Pezzulo AA, Gansemer ND, Samuel M, Prather RS, Zabner J, Fredericks DC, McCray PB Jr, Welsh MJ, Stoltz DA.
Proc Natl Acad Sci U S A. 2010 Nov 23;107(47):20571-5. doi: 10.1073/pnas.1015281107. Epub 2010 Nov 8.
PMID: 21059918

The air-liquid interface and use of primary cell cultures are important to recapitulate the transcriptional profile of in vivo airway epithelia. 
Pezzulo AA, Starner TD, Scheetz TE, Traver GL, Tilley AE, Harvey BG, Crystal RG, McCray PB Jr, Zabner J.
Am J Physiol Lung Cell Mol Physiol. 2011 Jan;300(1):L25-31. doi: 10.1152/ajplung.00256.2010. Epub 2010 Oct 22.
PMID: 20971803

Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. 
Meyerholz DK, Stoltz DA, Namati E, Ramachandran S, Pezzulo AA, Smith AR, Rector MV, Suter MJ, Kao S, McLennan G, Tearney GJ, Zabner J, McCray PB Jr, Welsh MJ.
Am J Respir Crit Care Med. 2010 Nov 15;182(10):1251-61. doi: 10.1164/rccm.201004-0643OC. Epub 2010 Jul 9.
PMID: 20622026

Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. 
Stoltz DA, Meyerholz DK, Pezzulo AA, Ramachandran S, Rogan MP, Davis GJ, Hanfland RA, Wohlford-Lenane C, Dohrn CL, Bartlett JA, Nelson GA 4th, Chang EH, Taft PJ, Ludwig PS, Estin M, Hornick EE, Launspach JL, Samuel M, Rokhlina T, Karp PH, Ostedgaard LS, Uc A, Starner TD, Horswill AR, Brogden KA, Prather RS, Richter SS, Shilyansky J, McCray PB Jr, Zabner J, Welsh MJ.
Sci Transl Med. 2010 Apr 28;2(29):29ra31. doi: 10.1126/scitranslmed.3000928.
PMID: 20427821

Isoform-specific regulation and localization of the coxsackie and adenovirus receptor in human airway epithelia. 
Excoffon KJ, Gansemer ND, Mobily ME, Karp PH, Parekh KR, Zabner J.
PLoS One. 2010 Mar 26;5(3):e9909. doi: 10.1371/journal.pone.0009909.
PMID: 20361046

Tryptase does not alter transepithelial conductance or paracellular permeability in human airway epithelial cells. 
Chang EH, Lee JH, Zabner J.
Am J Rhinol Allergy. 2010 Mar-Apr;24(2):126-8. doi: 10.2500/ajra.2010.24.3442.
PMID: 20338110

Paraoxonase 1, quorum sensing, and P. aeruginosa infection: a novel model. 
Estin ML, Stoltz DA, Zabner J.
Adv Exp Med Biol. 2010;660:183-93. doi: 10.1007/978-1-60761-350-3_17.
PMID: 20221881

A common mutation in paraoxonase-2 results in impaired lactonase activity. 
Stoltz DA, Ozer EA, Recker TJ, Estin M, Yang X, Shih DM, Lusis AJ, Zabner J.
J Biol Chem. 2009 Dec 18;284(51):35564-71. doi: 10.1074/jbc.M109.051706. Epub .
PMID: 19840942

Adenovirus 5-fiber 35 chimeric vector mediates efficient apical correction of the cystic fibrosis transmembrane conductance regulator defect in cystic fibrosis primary airway epithelia. 
Granio O, Ashbourne Excoffon KJ, Henning P, Melin P, Norez C, Gonzalez G, Karp PH, Magnusson MK, Habib N, Lindholm L, Becq F, Boulanger P, Zabner J, Hong SS.
Hum Gene Ther. 2010 Mar;21(3):251-69. doi: 10.1089/hum.2009.056.
PMID: 19788389

Patterns and density of early tracheal colonization in intensive care unit patients. 
Durairaj L, Mohamad Z, Launspach JL, Ashare A, Choi JY, Rajagopal S, Doern GV, Zabner J.
J Crit Care. 2009 Mar;24(1):114-21. doi: 10.1016/j.jcrc.2008.10.009.
PMID: 19272547

MMP9 modulates tight junction integrity and cell viability in human airway epithelia. 
Vermeer PD, Denker J, Estin M, Moninger TO, Keshavjee S, Karp P, Kline JN, Zabner J.
Am J Physiol Lung Cell Mol Physiol. 2009 May;296(5):L751-62. doi: 10.1152/ajplung.90578.2008. Epub 2009 Mar 6.
PMID: 19270179

Directed evolution of adeno-associated virus to an infectious respiratory virus. 
Excoffon KJ, Koerber JT, Dickey DD, Murtha M, Keshavjee S, Kaspar BK, Zabner J, Schaffer DV.
Proc Natl Acad Sci U S A. 2009 Mar 10;106(10):3865-70. doi: 10.1073/pnas.0813365106. Epub 2009 Feb 23.
PMID: 19237554

Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. 
Rogers CS, Stoltz DA, Meyerholz DK, Ostedgaard LS, Rokhlina T, Taft PJ, Rogan MP, Pezzulo AA, Karp PH, Itani OA, Kabel AC, Wohlford-Lenane CL, Davis GJ, Hanfland RA, Smith TL, Samuel M, Wax D, Murphy CN, Rieke A, Whitworth K, Uc A, Starner TD, Brogden KA, Shilyansky J, McCray PB Jr, Zabner J, Prather RS, Welsh MJ.
Science. 2008 Sep 26;321(5897):1837-41. doi: 10.1126/science.1163600.
PMID: 18818360

Drosophila are protected from Pseudomonas aeruginosa lethality by transgenic expression of paraoxonase-1. 
Stoltz DA, Ozer EA, Taft PJ, Barry M, Liu L, Kiss PJ, Moninger TO, Parsek MR, Zabner J.
J Clin Invest. 2008 Sep;118(9):3123-31. doi: 10.1172/JCI35147.
PMID: 18704198

The porcine lung as a potential model for cystic fibrosis. 
Rogers CS, Abraham WM, Brogden KA, Engelhardt JF, Fisher JT, McCray PB Jr, McLennan G, Meyerholz DK, Namati E, Ostedgaard LS, Prather RS, Sabater JR, Stoltz DA, Zabner J, Welsh MJ.
Am J Physiol Lung Cell Mol Physiol. 2008 Aug;295(2):L240-63. doi: 10.1152/ajplung.90203.2008. Epub 2008 May 16. Review.
PMID: 18487356

Reovirus preferentially infects the basolateral surface and is released from the apical surface of polarized human respiratory epithelial cells. 
Excoffon KJ, Guglielmi KM, Wetzel JD, Gansemer ND, Campbell JA, Dermody TS, Zabner J.
J Infect Dis. 2008 Apr 15;197(8):1189-97. doi: 10.1086/529515.
PMID: 18419529