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Lack of cystic fibrosis transmembrane conductance regulator disrupts fetal airway development in pigs.

Meyerholz DK, Stoltz DA, Gansemer ND, Ernst SE, Cook DP, Strub MD, LeClair EN, Barker CK, Adam RJ, Leidinger MR, Gibson-Corley KN, Karp PH, Welsh MJ, McCray PB Jr.

Lab Invest. 2018 Jun;98(6):825-838. doi: 10.1038/s41374-018-0026-7. Epub 2018 Feb 21.


Developing a platform system for gene delivery: amplifying virus-like particles (AVLP) as an influenza vaccine.

Wei H, Chen Z, Elson A, Li Z, Abraham M, Phan S, Kristhnamurthy S, McCray PB Jr, Andrews S, Stice S, Sakamoto K, Jones C, Tompkins SM, He B.

NPJ Vaccines. 2017 Nov 20;2:32. doi: 10.1038/s41541-017-0031-7. eCollection 2017.


Attenuation of pulmonary ACE2 activity impairs inactivation of des-Arg9 bradykinin/BKB1R axis and facilitates LPS-induced neutrophil infiltration.

Sodhi CP, Wohlford-Lenane C, Yamaguchi Y, Prindle T, Fulton WB, Wang S, McCray PB Jr, Chappell M, Hackam DJ, Jia H.

Am J Physiol Lung Cell Mol Physiol. 2018 Jan 1;314(1):L17-L31. doi: 10.1152/ajplung.00498.2016. Epub 2017 Sep 21.


Delayed neutrophil apoptosis enhances NET formation in cystic fibrosis.

Gray RD, Hardisty G, Regan KH, Smith M, Robb CT, Duffin R, Mackellar A, Felton JM, Paemka L, McCullagh BN, Lucas CD, Dorward DA, McKone EF, Cooke G, Donnelly SC, Singh PK, Stoltz DA, Haslett C, McCray PB, Whyte MKB, Rossi AG, Davidson DJ.

Thorax. 2018 Feb;73(2):134-144. doi: 10.1136/thoraxjnl-2017-210134. Epub 2017 Sep 15.


Long-term correction of hemophilia A mice following lentiviral mediated delivery of an optimized canine factor VIII gene.

Staber JM, Pollpeter MJ, Anderson CG, Burrascano M, Cooney AL, Sinn PL, Rutkowski DT, Raschke WC, McCray PB.

Gene Ther. 2017 Nov;24(11):742-748. doi: 10.1038/gt.2017.67. Epub 2017 Sep 14.


Novel GP64 envelope variants for improved delivery to human airway epithelial cells.

Sinn PL, Hwang BY, Li N, Ortiz JLS, Shirazi E, Parekh KR, Cooney AL, Schaffer DV, McCray PB Jr.

Gene Ther. 2017 Oct;24(10):674-679. doi: 10.1038/gt.2017.78. Epub 2017 Sep 7.


The tetraspanin CD9 facilitates MERS-coronavirus entry by scaffolding host cell receptors and proteases.

Earnest JT, Hantak MP, Li K, McCray PB Jr, Perlman S, Gallagher T.

PLoS Pathog. 2017 Jul 31;13(7):e1006546. doi: 10.1371/journal.ppat.1006546. eCollection 2017 Jul.


Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth.

Paemka L, McCullagh BN, Abou Alaiwa MH, Stoltz DA, Dong Q, Randak CO, Gray RD, McCray PB Jr.

J Cyst Fibros. 2017 Jul;16(4):471-474. doi: 10.1016/j.jcf.2017.03.007. Epub 2017 Apr 1.


Mouse-adapted MERS coronavirus causes lethal lung disease in human DPP4 knockin mice.

Li K, Wohlford-Lenane CL, Channappanavar R, Park JE, Earnest JT, Bair TB, Bates AM, Brogden KA, Flaherty HA, Gallagher T, Meyerholz DK, Perlman S, McCray PB Jr.

Proc Natl Acad Sci U S A. 2017 Apr 11;114(15):E3119-E3128. doi: 10.1073/pnas.1619109114. Epub 2017 Mar 27.


Proteolytic processing of Middle East respiratory syndrome coronavirus spikes expands virus tropism.

Park JE, Li K, Barlan A, Fehr AR, Perlman S, McCray PB Jr, Gallagher T.

Proc Natl Acad Sci U S A. 2016 Oct 25;113(43):12262-12267. Epub 2016 Oct 10.


SYVN1, NEDD8, and FBXO2 Proteins Regulate ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Ubiquitin-mediated Proteasomal Degradation.

Ramachandran S, Osterhaus SR, Parekh KR, Jacobi AM, Behlke MA, McCray PB Jr.

J Biol Chem. 2016 Dec 2;291(49):25489-25504. Epub 2016 Oct 18.


Lentiviral-mediated phenotypic correction of cystic fibrosis pigs.

Cooney AL, Abou Alaiwa MH, Shah VS, Bouzek DC, Stroik MR, Powers LS, Gansemer ND, Meyerholz DK, Welsh MJ, Stoltz DA, Sinn PL, McCray PB Jr.

JCI Insight. 2016 Sep 8;1(14). pii: e88730.


Novel Innate Immune Genes Regulating the Macrophage Response to Gram Positive Bacteria.

Alper S, Warg LA, De Arras L, Flatley BR, Davidson EJ, Adams J, Smith K, Wohlford-Lenane CL, McCray PB Jr, Pedersen BS, Schwartz DA, Yang IV.

Genetics. 2016 Sep;204(1):327-36. doi: 10.1534/genetics.115.185314. Epub 2016 Jun 29.


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.


Human, Pig, and Mouse Interferon-Induced Transmembrane Proteins Partially Restrict Pseudotyped Lentiviral Vectors.

Hornick AL, Li N, Oakland M, McCray PB Jr, Sinn PL.

Hum Gene Ther. 2016 May;27(5):354-62. doi: 10.1089/hum.2015.156.


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.


Dipeptidyl Peptidase 4 Distribution in the Human Respiratory Tract: Implications for the Middle East Respiratory Syndrome.

Meyerholz DK, Lambertz AM, McCray PB Jr.

Am J Pathol. 2016 Jan;186(1):78-86. doi: 10.1016/j.ajpath.2015.09.014. Epub 2015 Nov 18.


Immunohistochemical Detection of Markers for Translational Studies of Lung Disease in Pigs and Humans.

Meyerholz DK, Lambertz AM, Reznikov LR, Ofori-Amanfo GK, Karp PH, McCray PB Jr, Welsh MJ, Stoltz DA.

Toxicol Pathol. 2016 Apr;44(3):434-41. doi: 10.1177/0192623315609691. Epub 2015 Oct 27.


Highly differentiated human airway epithelial cells: a model to study host cell-parasite interactions in pertussis.

Guevara C, Zhang C, Gaddy JA, Iqbal J, Guerra J, Greenberg DP, Decker MD, Carbonetti N, Starner TD, McCray PB Jr, Mooi FR, Gómez-Duarte OG.

Infect Dis (Lond). 2016;48(3):177-88. doi: 10.3109/23744235.2015.1100323. Epub 2015 Oct 22.


Middle East Respiratory Syndrome Coronavirus Causes Multiple Organ Damage and Lethal Disease in Mice Transgenic for Human Dipeptidyl Peptidase 4.

Li K, Wohlford-Lenane C, Perlman S, Zhao J, Jewell AK, Reznikov LR, Gibson-Corley KN, Meyerholz DK, McCray PB Jr.

J Infect Dis. 2016 Mar 1;213(5):712-22. doi: 10.1093/infdis/jiv499. Epub 2015 Oct 20.


Increased susceptibility to otitis media in a Splunc1-deficient mouse model.

Bartlett JA, Meyerholz DK, Wohlford-Lenane CL, Naumann PW, Salzman NH, McCray PB Jr.

Dis Model Mech. 2015 May;8(5):501-8. doi: 10.1242/dmm.019646. Epub 2015 Mar 12.


The innate immune function of airway epithelial cells in inflammatory lung disease.

Hiemstra PS, McCray PB Jr, Bals R.

Eur Respir J. 2015 Apr;45(4):1150-62. doi: 10.1183/09031936.00141514. Epub 2015 Feb 19. Review.


Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy.

Yan Z, Stewart ZA, Sinn PL, Olsen JC, Hu J, McCray PB Jr, Engelhardt JF.

Hum Gene Ther Clin Dev. 2015 Mar;26(1):38-49. doi: 10.1089/humc.2014.154. Epub 2015 Feb 12. Review.


NETs and CF Lung Disease: Current Status and Future Prospects.

Gray RD, McCullagh BN, McCray PB.

Antibiotics (Basel). 2015 Jan 15;4(1):62-75. doi: 10.3390/antibiotics4010062. Review.


Platelet Activating Factor Receptor Activation Improves siRNA Uptake and RNAi Responses in Well-differentiated Airway Epithelia.

Krishnamurthy S, Behlke MA, Apicella MA, McCray PB Jr, Davidson BL.

Mol Ther Nucleic Acids. 2014 Jul 15;3:e175. doi: 10.1038/mtna.2014.26.


miR-31 dysregulation in cystic fibrosis airways contributes to increased pulmonary cathepsin S production.

Weldon S, McNally P, McAuley DF, Oglesby IK, Wohlford-Lenane CL, Bartlett JA, Scott CJ, McElvaney NG, Greene CM, McCray PB Jr, Taggart CC.

Am J Respir Crit Care Med. 2014 Jul 15;190(2):165-74. doi: 10.1164/rccm.201311-1986OC.


A genomic signature approach to rescue ΔF508-cystic fibrosis transmembrane conductance regulator biosynthesis and function.

Ramachandran S, Osterhaus SR, Karp PH, Welsh MJ, McCray PB Jr.

Am J Respir Cell Mol Biol. 2014 Sep;51(3):354-62. doi: 10.1165/rcmb.2014-0007OC.


Rapid generation of a mouse model for Middle East respiratory syndrome.

Zhao J, Li K, Wohlford-Lenane C, Agnihothram SS, Fett C, Zhao J, Gale MJ Jr, Baric RS, Enjuanes L, Gallagher T, McCray PB Jr, Perlman S.

Proc Natl Acad Sci U S A. 2014 Apr 1;111(13):4970-5. doi: 10.1073/pnas.1323279111. Epub 2014 Mar 5.


Effects of airway surface liquid pH on host defense in cystic fibrosis.

Berkebile AR, McCray PB Jr.

Int J Biochem Cell Biol. 2014 Jul;52:124-9. doi: 10.1016/j.biocel.2014.02.009. Epub 2014 Feb 19. Review.


Receptor variation and susceptibility to Middle East respiratory syndrome coronavirus infection.

Barlan A, Zhao J, Sarkar MK, Li K, McCray PB Jr, Perlman S, Gallagher T.

J Virol. 2014 May;88(9):4953-61. doi: 10.1128/JVI.00161-14. Epub 2014 Feb 19.


piggyBac-mediated phenotypic correction of factor VIII deficiency.

Staber JM, Pollpeter MJ, Arensdorf A, Sinn PL, Rutkowski DT, McCray PB Jr.

Mol Ther Methods Clin Dev. 2014 Sep 10;1:14042. doi: 10.1038/mtm.2014.42. eCollection 2014.


A novel gene delivery method transduces porcine pancreatic duct epithelial cells.

Griffin MA, Restrepo MS, Abu-El-Haija M, Wallen T, Buchanan E, Rokhlina T, Chen YH, McCray PB Jr, Davidson BL, Divekar A, Uc A.

Gene Ther. 2014 Feb;21(2):123-30. doi: 10.1038/gt.2013.62. Epub 2013 Nov 21.


Genotype-specific alterations in vascular smooth muscle cell function in cystic fibrosis piglets.

Guo JJ, Stoltz DA, Zhu V, Volk KA, Segar JL, McCray PB Jr, Roghair RD.

J Cyst Fibros. 2014 May;13(3):251-9. doi: 10.1016/j.jcf.2013.10.009. Epub 2013 Oct 31.


Tracheomalacia is associated with lower FEV1 and Pseudomonas acquisition in children with CF.

Fischer AJ, Singh SB, Adam RJ, Stoltz DA, Baranano CF, Kao S, Weinberger MM, McCray PB Jr, Starner TD.

Pediatr Pulmonol. 2014 Oct;49(10):960-70. doi: 10.1002/ppul.22922. Epub 2013 Oct 25.


Increased concentration of iodide in airway secretions is associated with reduced respiratory syncytial virus disease severity.

Derscheid RJ, van Geelen A, Berkebile AR, Gallup JM, Hostetter SJ, Banfi B, McCray PB Jr, Ackermann MR.

Am J Respir Cell Mol Biol. 2014 Feb;50(2):389-97. doi: 10.1165/rcmb.2012-0529OC.


Person-to-person spread of the MERS coronavirus--an evolving picture.

Perlman S, McCray PB Jr.

N Engl J Med. 2013 Aug 1;369(5):466-7. doi: 10.1056/NEJMe1308724. No abstract available.


piggyBac transposase tools for genome engineering.

Li X, Burnight ER, Cooney AL, Malani N, Brady T, Sander JD, Staber J, Wheelan SJ, Joung JK, McCray PB Jr, Bushman FD, Sinn PL, Craig NL.

Proc Natl Acad Sci U S A. 2013 Jun 18;110(25):E2279-87. doi: 10.1073/pnas.1305987110. Epub 2013 May 30.


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.


Post-transcriptional regulation of cystic fibrosis transmembrane conductance regulator expression and function by microRNAs.

Ramachandran S, Karp PH, Osterhaus SR, Jiang P, Wohlford-Lenane C, Lennox KA, Jacobi AM, Praekh K, Rose SD, Behlke MA, Xing Y, Welsh MJ, McCray PB Jr.

Am J Respir Cell Mol Biol. 2013 Oct;49(4):544-51. doi: 10.1165/rcmb.2012-0430OC.


Efficient delivery of RNA interference oligonucleotides to polarized airway epithelia in vitro.

Ramachandran S, Krishnamurthy S, Jacobi AM, Wohlford-Lenane C, Behlke MA, Davidson BL, McCray PB Jr.

Am J Physiol Lung Cell Mol Physiol. 2013 Jul 1;305(1):L23-32. doi: 10.1152/ajplung.00426.2012. Epub 2013 Apr 26.


SPLUNC1/BPIFA1 contributes to pulmonary host defense against Klebsiella pneumoniae respiratory infection.

Liu Y, Bartlett JA, Di ME, Bomberger JM, Chan YR, Gakhar L, Mallampalli RK, McCray PB Jr, Di YP.

Am J Pathol. 2013 May;182(5):1519-31. doi: 10.1016/j.ajpath.2013.01.050. Epub 2013 Mar 15.


Intrapulmonary Versus Nasal Transduction of Murine Airways With GP64-pseudotyped Viral Vectors.

Oakland M, Maury W, McCray PB Jr, Sinn PL.

Mol Ther Nucleic Acids. 2013 Jan 29;2:e69. doi: 10.1038/mtna.2012.60.


A Hyperactive Transposase Promotes Persistent Gene Transfer of a piggyBac DNA Transposon.

Burnight ER, Staber JM, Korsakov P, Li X, Brett BT, Scheetz TE, Craig NL, McCray PB Jr.

Mol Ther Nucleic Acids. 2012 Oct 16;1:e50. doi: 10.1038/mtna.2012.12.


Manipulation of Cell Physiology Enables Gene Silencing in Well-differentiated Airway Epithelia.

Krishnamurthy S, Behlke MA, Ramachandran S, Salem AK, McCray PB Jr, Davidson BL.

Mol Ther Nucleic Acids. 2012 Aug 28;1:e41. doi: 10.1038/mtna.2012.36.


Lentiviral vector gene transfer to porcine airways.

Sinn PL, Cooney AL, Oakland M, Dylla DE, Wallen TJ, Pezzulo AA, Chang EH, McCray PB Jr.

Mol Ther Nucleic Acids. 2012 Nov 27;1:e56. doi: 10.1038/mtna.2012.47.


Intranasal treatment with poly(I•C) protects aged mice from lethal respiratory virus infections.

Zhao J, Wohlford-Lenane C, Zhao J, Fleming E, Lane TE, McCray PB Jr, Perlman S.

J Virol. 2012 Nov;86(21):11416-24. doi: 10.1128/JVI.01410-12. Epub 2012 Aug 22.


A microRNA network regulates expression and biosynthesis of wild-type and DeltaF508 mutant cystic fibrosis transmembrane conductance regulator.

Ramachandran S, Karp PH, Jiang P, Ostedgaard LS, Walz AE, Fisher JT, Keshavjee S, Lennox KA, Jacobi AM, Rose SD, Behlke MA, Welsh MJ, Xing Y, McCray PB Jr.

Proc Natl Acad Sci U S A. 2012 Aug 14;109(33):13362-7. doi: 10.1073/pnas.1210906109. Epub 2012 Aug 1.


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.

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