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Items: 43

1.

Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia.

Muraglia KA, Chorghade RS, Kim BR, Tang XX, Shah VS, Grillo AS, Daniels PN, Cioffi AG, Karp PH, Zhu L, Welsh MJ, Burke MD.

Nature. 2019 Mar;567(7748):405-408. doi: 10.1038/s41586-019-1018-5. Epub 2019 Mar 13.

2.

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.

3.

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.

4.

Motile cilia of human airway epithelia contain hedgehog signaling components that mediate noncanonical hedgehog signaling.

Mao S, Shah AS, Moninger TO, Ostedgaard LS, Lu L, Tang XX, Thornell IM, Reznikov LR, Ernst SE, Karp PH, Tan P, Keshavjee S, Abou Alaiwa MH, Welsh MJ.

Proc Natl Acad Sci U S A. 2018 Feb 6;115(6):1370-1375. doi: 10.1073/pnas.1719177115. Epub 2018 Jan 22.

5.

Relationships among CFTR expression, HCO3- secretion, and host defense may inform gene- and cell-based cystic fibrosis therapies.

Shah VS, Ernst S, Tang XX, Karp PH, Parker CP, Ostedgaard LS, Welsh MJ.

Proc Natl Acad Sci U S A. 2016 May 10;113(19):5382-7. doi: 10.1073/pnas.1604905113. Epub 2016 Apr 25.

6.

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.

7.

Acidic pH increases airway surface liquid viscosity in cystic fibrosis.

Tang XX, Ostedgaard LS, Hoegger MJ, Moninger TO, Karp PH, McMenimen JD, Choudhury B, Varki A, Stoltz DA, Welsh MJ.

J Clin Invest. 2016 Mar 1;126(3):879-91. doi: 10.1172/JCI83922. Epub 2016 Jan 25.

8.

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.

9.

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.

10.

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.

11.

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.

12.

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.

13.

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.

14.

Chemosensory functions for pulmonary neuroendocrine cells.

Gu X, Karp PH, Brody SL, Pierce RA, Welsh MJ, Holtzman MJ, Ben-Shahar Y.

Am J Respir Cell Mol Biol. 2014 Mar;50(3):637-46. doi: 10.1165/rcmb.2013-0199OC.

15.

Multicenter intestinal current measurements in rectal biopsies from CF and non-CF subjects to monitor CFTR function.

Clancy JP, Szczesniak RD, Ashlock MA, Ernst SE, Fan L, Hornick DB, Karp PH, Khan U, Lymp J, Ostmann AJ, Rezayat A, Starner TD, Sugandha SP, Sun H, Quinney N, Donaldson SH, Rowe SM, Gabriel SE.

PLoS One. 2013 Sep 10;8(9):e73905. doi: 10.1371/journal.pone.0073905. eCollection 2013.

16.

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.

17.

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.

18.

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.

19.

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.

20.

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.

21.

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.

22.

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.

23.

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.

24.

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.

25.

Cystic fibrosis transmembrane conductance regulator with a shortened R domain rescues the intestinal phenotype of CFTR-/- mice.

Ostedgaard LS, Meyerholz DK, Vermeer DW, Karp PH, Schneider L, Sigmund CD, Welsh MJ.

Proc Natl Acad Sci U S A. 2011 Feb 15;108(7):2921-6. doi: 10.1073/pnas.1019752108. Epub 2011 Feb 1.

26.

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.

27.

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.

28.

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.

29.

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
30.

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.

31.

Processing and function of CFTR-DeltaF508 are species-dependent.

Ostedgaard LS, Rogers CS, Dong Q, Randak CO, Vermeer DW, Rokhlina T, Karp PH, Welsh MJ.

Proc Natl Acad Sci U S A. 2007 Sep 25;104(39):15370-5. Epub 2007 Sep 14.

32.

Cellular localization and activity of Ad-delivered GFP-CFTR in airway epithelial and tracheal cells.

Granio O, Norez C, Ashbourne Excoffon KJ, Karp PH, Lusky M, Becq F, Boulanger P, Zabner J, Hong SS.

Am J Respir Cell Mol Biol. 2007 Dec;37(6):631-9. Epub 2007 Jul 19.

PMID:
17641299
33.

Gene transfer of CFTR to airway epithelia: low levels of expression are sufficient to correct Cl- transport and overexpression can generate basolateral CFTR.

Farmen SL, Karp PH, Ng P, Palmer DJ, Koehler DR, Hu J, Beaudet AL, Zabner J, Welsh MJ.

Am J Physiol Lung Cell Mol Physiol. 2005 Dec;289(6):L1123-30. Epub 2005 Aug 5.

34.

A shortened adeno-associated virus expression cassette for CFTR gene transfer to cystic fibrosis airway epithelia.

Ostedgaard LS, Rokhlina T, Karp PH, Lashmit P, Afione S, Schmidt M, Zabner J, Stinski MF, Chiorini JA, Welsh MJ.

Proc Natl Acad Sci U S A. 2005 Feb 22;102(8):2952-7. Epub 2005 Feb 9.

35.

Curcumin stimulates cystic fibrosis transmembrane conductance regulator Cl- channel activity.

Berger AL, Randak CO, Ostedgaard LS, Karp PH, Vermeer DW, Welsh MJ.

J Biol Chem. 2005 Feb 18;280(7):5221-6. Epub 2004 Dec 6.

36.

Thixotropic solutions enhance viral-mediated gene transfer to airway epithelia.

Seiler MP, Luner P, Moninger TO, Karp PH, Keshavjee S, Zabner J.

Am J Respir Cell Mol Biol. 2002 Aug;27(2):133-40.

PMID:
12151304
37.

An in vitro model of differentiated human airway epithelia. Methods for establishing primary cultures.

Karp PH, Moninger TO, Weber SP, Nesselhauf TS, Launspach JL, Zabner J, Welsh MJ.

Methods Mol Biol. 2002;188:115-37. No abstract available.

PMID:
11987537
38.

CFTR with a partially deleted R domain corrects the cystic fibrosis chloride transport defect in human airway epithelia in vitro and in mouse nasal mucosa in vivo.

Ostedgaard LS, Zabner J, Vermeer DW, Rokhlina T, Karp PH, Stecenko AA, Randak C, Welsh MJ.

Proc Natl Acad Sci U S A. 2002 Mar 5;99(5):3093-8. Epub 2002 Feb 19.

39.

KGF alters gene expression in human airway epithelia: potential regulation of the inflammatory response.

Prince LS, Karp PH, Moninger TO, Welsh MJ.

Physiol Genomics. 2001 Jul 17;6(2):81-9.

PMID:
11459923
40.

Effect of topical nasal pharmaceuticals on sodium and chloride transport by human airway epithelia.

Jepsen M, Graham S, Karp PH, Zabner J.

Am J Rhinol. 2000 Nov-Dec;14(6):405-9.

PMID:
11197117
41.

The osmolyte xylitol reduces the salt concentration of airway surface liquid and may enhance bacterial killing.

Zabner J, Seiler MP, Launspach JL, Karp PH, Kearney WR, Look DC, Smith JJ, Welsh MJ.

Proc Natl Acad Sci U S A. 2000 Oct 10;97(21):11614-9.

42.

Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.

Zabner J, Smith JJ, Karp PH, Widdicombe JH, Welsh MJ.

Mol Cell. 1998 Sep;2(3):397-403.

43.

Defective fluid transport by cystic fibrosis airway epithelia.

Smith JJ, Karp PH, Welsh MJ.

J Clin Invest. 1994 Mar;93(3):1307-11.

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