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The Effects of Timed Light Exposure in Critically Ill Patients: A Randomized Controlled Pilot Study.

Gehlbach BK, Patel SB, Van Cauter E, Pohlman AS, Hall JB, Zabner J.

Am J Respir Crit Care Med. 2018 Mar 12. doi: 10.1164/rccm.201801-0170LE. [Epub ahead of print] No abstract available.


Airway surface liquid from smokers promotes bacterial growth and biofilm formation via iron-lactoferrin imbalance.

Vargas Buonfiglio LG, Borcherding JA, Frommelt M, Parker GJ, Duchman B, Vanegas Calderón OG, Fernandez-Ruiz R, Noriega JE, Stone EA, Gerke AK, Zabner J, Comellas AP.

Respir Res. 2018 Mar 10;19(1):42. doi: 10.1186/s12931-018-0743-x.



O'Malley Y, Rotti PG, Thornell IM, Vanegas Calderón OG, Febres-Aldana C, Durham K, Yao J, Li X, Zhu Z, Norris AW, Zabner J, Engelhardt JF, Uc A.

J Appl Physiol (1985). 2018 Mar 8. doi: 10.1152/japplphysiol.00043.2018. [Epub ahead of print]


Nominal carbonic anhydrase activity minimizes airway-surface liquid pH changes during breathing.

Thornell IM, Li X, Tang XX, Brommel CM, Karp PH, Welsh MJ, Zabner J.

Physiol Rep. 2018 Jan;6(2). doi: 10.14814/phy2.13569.


Chest wall strapping increases expiratory airflow and detectable airway segments in computer tomographic scans of normal and obstructed lungs.

Taher H, Bauer C, Abston E, Kaczka DW, Bhatt SP, Zabner J, Brower RG, Beichel RR, Eberlein M.

J Appl Physiol (1985). 2018 Jan 4. doi: 10.1152/japplphysiol.00184.2017. [Epub ahead of print]


Higher BMI is associated with higher expiratory airflow normalised for lung volume (FEF25-75/FVC) in COPD.

Abston E, Comellas A, Reed RM, Kim V, Wise RA, Brower R, Fortis S, Beichel R, Bhatt S, Zabner J, Newell J, Hoffman EA, Eberlein M.

BMJ Open Respir Res. 2017 Oct 13;4(1):e000231. doi: 10.1136/bmjresp-2017-000231. eCollection 2017.


Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.

Li X, Vargas Buonfiglio LG, Adam RJ, Stoltz DA, Zabner J, Comellas AP.

Crit Care Med. 2017 Dec;45(12):e1240-e1246. doi: 10.1097/CCM.0000000000002720.


Effect of vitamin D3 on the antimicrobial activity of human airway surface liquid: preliminary results of a randomised placebo-controlled double-blind trial.

Vargas Buonfiglio LG, Cano M, Pezzulo AA, Vanegas Calderon OG, Zabner J, Gerke AK, Comellas AP.

BMJ Open Respir Res. 2017 Jun 4;4(1):e000211. doi: 10.1136/bmjresp-2017-000211. eCollection 2017.


Effects of Coal Fly Ash Particulate Matter on the Antimicrobial Activity of Airway Surface Liquid.

Vargas Buonfiglio LG, Mudunkotuwa IA, Abou Alaiwa MH, Vanegas Calderón OG, Borcherding JA, Gerke AK, Zabner J, Grassian VH, Comellas AP.

Environ Health Perspect. 2017 Jul 5;125(7):077003. doi: 10.1289/EHP876.


Postnatal airway growth in cystic fibrosis piglets.

Adam RJ, Abou Alaiwa MH, Bouzek DC, Cook DP, Gansemer ND, Taft PJ, Powers LS, Stroik MR, Hoegger MJ, McMenimen JD, Hoffman EA, Zabner J, Welsh MJ, Meyerholz DK, Stoltz DA.

J Appl Physiol (1985). 2017 Sep 1;123(3):526-533. doi: 10.1152/japplphysiol.00263.2017. Epub 2017 Jun 15.


CFTR gene transfer with AAV improves early cystic fibrosis pig phenotypes.

Steines B, Dickey DD, Bergen J, Excoffon KJ, Weinstein JR, Li X, Yan Z, Abou Alaiwa MH, Shah VS, Bouzek DC, Powers LS, Gansemer ND, Ostedgaard LS, Engelhardt JF, Stoltz DA, Welsh MJ, Sinn PL, Schaffer DV, Zabner J.

JCI Insight. 2016 Sep 8;1(14):e88728.


Protein Kinase Cζ Inhibitor Promotes Resolution of Bleomycin-Induced Acute Lung Injury.

Buonfiglio LG, Bagegni M, Borcherding JA, Sieren JC, Caraballo JC, Reger A, Zabner J, Li X, Comellas AP.

Am J Respir Cell Mol Biol. 2016 Dec;55(6):869-877.


Repurposing tromethamine as inhaled therapy to treat CF airway disease.

Abou Alaiwa MH, Launspach JL, Sheets KA, Rivera JA, Gansemer ND, Taft PJ, Thorne PS, Welsh MJ, Stoltz DA, Zabner J.

JCI Insight. 2016 Jun 2;1(8). pii: e87535.


Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus.

Bartlett JA, Ramachandran S, Wohlford-Lenane CL, Barker CK, Pezzulo AA, Zabner J, Welsh MJ, Meyerholz DK, Stoltz DA, McCray PB Jr.

Am J Respir Crit Care Med. 2016 Oct 1;194(7):845-854.


Loss of carbonic anhydrase XII function in individuals with elevated sweat chloride concentration and pulmonary airway disease.

Lee M, Vecchio-Pagán B, Sharma N, Waheed A, Li X, Raraigh KS, Robbins S, Han ST, Franca AL, Pellicore MJ, Evans TA, Arcara KM, Nguyen H, Luan S, Belchis D, Hertecant J, Zabner J, Sly WS, Cutting GR.

Hum Mol Genet. 2016 May 15;25(10):1923-1933. Epub 2016 Feb 23.


Airway acidification initiates host defense abnormalities in cystic fibrosis mice.

Shah VS, Meyerholz DK, Tang XX, Reznikov L, Abou Alaiwa M, Ernst SE, Karp PH, Wohlford-Lenane CL, Heilmann KP, Leidinger MR, Allen PD, Zabner J, McCray PB Jr, Ostedgaard LS, Stoltz DA, Randak CO, Welsh MJ.

Science. 2016 Jan 29;351(6272):503-7. doi: 10.1126/science.aad5589.


Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense.

Li X, Tang XX, Vargas Buonfiglio LG, Comellas AP, Thornell IM, Ramachandran S, Karp PH, Taft PJ, Sheets K, Abou Alaiwa MH, Welsh MJ, Meyerholz DK, Stoltz DA, Zabner J.

Am J Physiol Lung Cell Mol Physiol. 2016 Apr 1;310(7):L670-9. doi: 10.1152/ajplung.00422.2015. Epub 2016 Jan 22.


Whole exome sequencing identifies novel candidate genes that modify chronic obstructive pulmonary disease susceptibility.

Bruse S, Moreau M, Bromberg Y, Jang JH, Wang N, Ha H, Picchi M, Lin Y, Langley RJ, Qualls C, Klensney-Tait J, Zabner J, Leng S, Mao J, Belinsky SA, Xing J, Nyunoya T.

Hum Genomics. 2016 Jan 7;10:1. doi: 10.1186/s40246-015-0058-7.


Precision Genomic Medicine in Cystic Fibrosis.

Chang EH, Zabner J.

Clin Transl Sci. 2015 Oct;8(5):606-10. doi: 10.1111/cts.12292. Epub 2015 Jun 15. Review.


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 Oct 15;43(18):e116. doi: 10.1093/nar/gkv562. Epub 2015 Jun 3.


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):1435-1443. doi: 10.1378/chest.14-2241.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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, Abou 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.


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.


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.


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.


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.


Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung.

Pezzulo AA, Tang XX, Hoegger MJ, Abou 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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.


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.

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