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Sweat rate analysis of ivacaftor potentiation of CFTR in non-CF adults.

Kim J, Farahmand M, Dunn C, Milla CE, Horii RI, Thomas EAC, Moss RB, Wine JJ.

Sci Rep. 2018 Nov 2;8(1):16233. doi: 10.1038/s41598-018-34308-8.


How to live a long and healthy life with cystic fibrosis: Lessons from the CF ferret.

Wine JJ.

J Cyst Fibros. 2019 Jan;18(1):8-9. doi: 10.1016/j.jcf.2018.10.005. Epub 2018 Oct 23. No abstract available.


Granule-stored MUC5B mucins are packed by the non-covalent formation of N-terminal head-to-head tetramers.

Trillo-Muyo S, Nilsson HE, Recktenwald CV, Ermund A, Ridley C, Meiss LN, Bähr A, Klymiuk N, Wine JJ, Koeck PJB, Thornton DJ, Hebert H, Hansson GC.

J Biol Chem. 2018 Apr 13;293(15):5746-5754. doi: 10.1074/jbc.RA117.001014. Epub 2018 Feb 13.


Impaired PGE2-stimulated Cl- and HCO3- secretion contributes to cystic fibrosis airway disease.

Sellers ZM, Illek B, Figueira MF, Hari G, Joo NS, Sibley E, Souza-Menezes J, Morales MM, Fischer H, Wine JJ.

PLoS One. 2017 Dec 27;12(12):e0189894. doi: 10.1371/journal.pone.0189894. eCollection 2017.


Ivacaftor restores CFTR-dependent sweat gland fluid secretion in cystic fibrosis subjects with S945L alleles.

Kim J, Davies Z, Dunn C, Wine JJ, Milla C.

J Cyst Fibros. 2018 Mar;17(2):179-185. doi: 10.1016/j.jcf.2017.12.005. Epub 2017 Dec 24.


Progress in understanding mucus abnormalities in cystic fibrosis airways.

Wine JJ, Hansson GC, König P, Joo NS, Ermund A, Pieper M.

J Cyst Fibros. 2018 Mar;17(2S):S35-S39. doi: 10.1016/j.jcf.2017.09.003. Epub 2017 Sep 23. Review.


The normal trachea is cleaned by MUC5B mucin bundles from the submucosal glands coated with the MUC5AC mucin.

Ermund A, Meiss LN, Rodriguez-Pineiro AM, Bähr A, Nilsson HE, Trillo-Muyo S, Ridley C, Thornton DJ, Wine JJ, Hebert H, Klymiuk N, Hansson GC.

Biochem Biophys Res Commun. 2017 Oct 21;492(3):331-337. doi: 10.1016/j.bbrc.2017.08.113. Epub 2017 Aug 30.


The magnitude of ivacaftor effects on fluid secretion via R117H-CFTR channels: Human in vivo measurements.

Char JE, Dunn C, Davies Z, Milla C, Moss RB, Wine JJ.

PLoS One. 2017 Apr 18;12(4):e0175486. doi: 10.1371/journal.pone.0175486. eCollection 2017.


Evaporimeter and Bubble-Imaging Measures of Sweat Gland Secretion Rates.

Kim J, Farahmand M, Dunn C, Davies Z, Frisbee E, Milla C, Wine JJ.

PLoS One. 2016 Oct 21;11(10):e0165254. doi: 10.1371/journal.pone.0165254. eCollection 2016.


Inhibition of airway surface fluid absorption by cholinergic stimulation.

Joo NS, Krouse ME, Choi JY, Cho HJ, Wine JJ.

Sci Rep. 2016 Feb 5;6:20735. doi: 10.1038/srep20735.


Secretion rates of human nasal submucosal glands from patients with chronic rhinosinusitis or cystic fibrosis.

Jeong JH, Hwang PH, Cho DY, Joo NS, Wine JJ.

Am J Rhinol Allergy. 2015 Sep-Oct;29(5):334-8. doi: 10.2500/ajra.2015.29.4213.


Airway Gland Structure and Function.

Widdicombe JH, Wine JJ.

Physiol Rev. 2015 Oct;95(4):1241-319. doi: 10.1152/physrev.00039.2014. Review.


Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance.

Evans TI, Joo NS, Keiser NW, Yan Z, Tyler SR, Xie W, Zhang Y, Hsiao JJ, Cho HJ, Wright ME, Wine JJ, Engelhardt JF.

Am J Respir Cell Mol Biol. 2016 Apr;54(4):469-81. doi: 10.1165/rcmb.2015-0090OC.


Proteomic analysis of pure human airway gland mucus reveals a large component of protective proteins.

Joo NS, Evans IA, Cho HJ, Park IH, Engelhardt JF, Wine JJ.

PLoS One. 2015 Feb 23;10(2):e0116756. doi: 10.1371/journal.pone.0116756. eCollection 2015.


Measuring mucociliary transport and mucus properties in multiple regions of airway epithelial surfaces helps clarify cystic fibrosis defects.

Wine JJ.

Am J Respir Crit Care Med. 2014 Aug 15;190(4):364-5. doi: 10.1164/rccm.201407-1247ED. No abstract available.


Medicine. Letting go of mucus.

Wine JJ.

Science. 2014 Aug 15;345(6198):730-1. doi: 10.1126/science.1258493. No abstract available.


Mucociliary clearance and submucosal gland secretion in the ex vivo ferret trachea.

Jeong JH, Joo NS, Hwang PH, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2014 Jul 1;307(1):L83-93. doi: 10.1152/ajplung.00009.2014. Epub 2014 May 2.


A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor.

Char JE, Wolfe MH, Cho HJ, Park IH, Jeong JH, Frisbee E, Dunn C, Davies Z, Milla C, Moss RB, Thomas EA, Wine JJ.

PLoS One. 2014 Feb 10;9(2):e88564. doi: 10.1371/journal.pone.0088564. eCollection 2014.


In vivo readout of CFTR function: ratiometric measurement of CFTR-dependent secretion by individual, identifiable human sweat glands.

Wine JJ, Char JE, Chen J, Cho HJ, Dunn C, Frisbee E, Joo NS, Milla C, Modlin SE, Park IH, Thomas EA, Tran KV, Verma R, Wolfe MH.

PLoS One. 2013 Oct 24;8(10):e77114. doi: 10.1371/journal.pone.0077114. eCollection 2013. Erratum in: PLoS One. 2014;9(9):e109227.


Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809.

Van Goor F, Hadida S, Grootenhuis PD, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu PA.

Proc Natl Acad Sci U S A. 2011 Nov 15;108(46):18843-8. doi: 10.1073/pnas.1105787108. Epub 2011 Oct 5.


Defective fluid secretion from submucosal glands of nasal turbinates from CFTR-/- and CFTR (ΔF508/ΔF508) pigs.

Cho HJ, Joo NS, Wine JJ.

PLoS One. 2011;6(8):e24424. doi: 10.1371/journal.pone.0024424. Epub 2011 Aug 31.


Measurement of fluid secretion from intact airway submucosal glands.

Wine JJ, Joo NS, Choi JY, Cho HJ, Krouse ME, Wu JV, Khansaheb M, Irokawa T, Ianowski J, Hanrahan JW, Cuthbert AW, Tran KV.

Methods Mol Biol. 2011;742:93-112. doi: 10.1007/978-1-61779-120-8_6.


Properties of substance P-stimulated mucus secretion from porcine tracheal submucosal glands.

Khansaheb M, Choi JY, Joo NS, Yang YM, Krouse M, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2011 Mar;300(3):L370-9. doi: 10.1152/ajplung.00372.2010. Epub 2010 Dec 3.


Hyposecretion of fluid from tracheal submucosal glands of CFTR-deficient pigs.

Joo NS, Cho HJ, Khansaheb M, Wine JJ.

J Clin Invest. 2010 Sep;120(9):3161-6. doi: 10.1172/JCI43466. Epub 2010 Aug 25.


Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis.

Sun X, Sui H, Fisher JT, Yan Z, Liu X, Cho HJ, Joo NS, Zhang Y, Zhou W, Yi Y, Kinyon JM, Lei-Butters DC, Griffin MA, Naumann P, Luo M, Ascher J, Wang K, Frana T, Wine JJ, Meyerholz DK, Engelhardt JF.

J Clin Invest. 2010 Sep;120(9):3149-60. doi: 10.1172/JCI43052. Epub 2010 Aug 25.


Mucus secretion from individual submucosal glands of the ferret trachea.

Cho HJ, Joo NS, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2010 Jul;299(1):L124-36. doi: 10.1152/ajplung.00049.2010. Epub 2010 Apr 30.


The development of lung disease in cystic fibrosis pigs.

Wine JJ.

Sci Transl Med. 2010 Apr 28;2(29):29ps20. doi: 10.1126/scitranslmed.3001130.


Compartmentalized cyclic adenosine 3',5'-monophosphate at the plasma membrane clusters PDE3A and cystic fibrosis transmembrane conductance regulator into microdomains.

Penmatsa H, Zhang W, Yarlagadda S, Li C, Conoley VG, Yue J, Bahouth SW, Buddington RK, Zhang G, Nelson DJ, Sonecha MD, Manganiello V, Wine JJ, Naren AP.

Mol Biol Cell. 2010 Mar 15;21(6):1097-110. doi: 10.1091/mbc.E09-08-0655. Epub 2010 Jan 20.


Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770.

Van Goor F, Hadida S, Grootenhuis PD, Burton B, Cao D, Neuberger T, Turnbull A, Singh A, Joubran J, Hazlewood A, Zhou J, McCartney J, Arumugam V, Decker C, Yang J, Young C, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu P.

Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30. doi: 10.1073/pnas.0904709106. Epub 2009 Oct 21.


Substance P stimulates human airway submucosal gland secretion mainly via a CFTR-dependent process.

Choi JY, Khansaheb M, Joo NS, Krouse ME, Robbins RC, Weill D, Wine JJ.

J Clin Invest. 2009 May;119(5):1189-200. doi: 10.1172/JCI37284. Epub 2009 Apr 20. Erratum in: J Clin Invest. 2010 Mar 1;120(3):931-2.


Lubiprostone stimulates secretion from tracheal submucosal glands of sheep, pigs, and humans.

Joo NS, Wine JJ, Cuthbert AW.

Am J Physiol Lung Cell Mol Physiol. 2009 May;296(5):L811-24. doi: 10.1152/ajplung.90636.2008. Epub 2009 Feb 20.


Substance P stimulates CFTR-dependent fluid secretion by mouse tracheal submucosal glands.

Ianowski JP, Choi JY, Wine JJ, Hanrahan JW.

Pflugers Arch. 2008 Nov;457(2):529-37. doi: 10.1007/s00424-008-0527-0. Epub 2008 May 29.


Synergistic airway gland mucus secretion in response to vasoactive intestinal peptide and carbachol is lost in cystic fibrosis.

Choi JY, Joo NS, Krouse ME, Wu JV, Robbins RC, Ianowski JP, Hanrahan JW, Wine JJ.

J Clin Invest. 2007 Oct;117(10):3118-27.


The inexhaustible mouse nose. Focus on "olfactory epithelia exhibit progressive functional and morphological defects in CF mice".

Wine JJ.

Am J Physiol Cell Physiol. 2007 Aug;293(2):C537-9. Epub 2007 May 16. No abstract available.


Mucus secretion by single tracheal submucosal glands from normal and cystic fibrosis transmembrane conductance regulator knockout mice.

Ianowski JP, Choi JY, Wine JJ, Hanrahan JW.

J Physiol. 2007 Apr 1;580(Pt 1):301-14. Epub 2007 Jan 4.


Acinar origin of CFTR-dependent airway submucosal gland fluid secretion.

Wu JV, Krouse ME, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2007 Jan;292(1):L304-11. Epub 2006 Sep 22.


Acid in the airways. Focus on "Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis".

Wine JJ.

Am J Physiol Cell Physiol. 2006 Mar;290(3):C669-71. No abstract available.


Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands.

Joo NS, Irokawa T, Robbins RC, Wine JJ.

J Biol Chem. 2006 Mar 17;281(11):7392-8. Epub 2006 Jan 12.


Submucosal glands and airway defense.

Wine JJ, Joo NS.

Proc Am Thorac Soc. 2004;1(1):47-53. Review.


An inwardly rectifying potassium channel in apical membrane of Calu-3 cells.

Wu JV, Krouse ME, Rustagi A, Joo NS, Wine JJ.

J Biol Chem. 2004 Nov 5;279(45):46558-65. Epub 2004 Aug 24.


Acid and base secretion in the Calu-3 model of human serous cells.

Krouse ME, Talbott JF, Lee MM, Joo NS, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2004 Dec;287(6):L1274-83. Epub 2004 Aug 13.


Regulation of antiprotease and antimicrobial protein secretion by airway submucosal gland serous cells.

Joo NS, Lee DJ, Winges KM, Rustagi A, Wine JJ.

J Biol Chem. 2004 Sep 10;279(37):38854-60. Epub 2004 Jul 2.


A "virtual gland" method for quantifying epithelial fluid secretion.

Irokawa T, Krouse ME, Joo NS, Wu JV, Wine JJ.

Am J Physiol Lung Cell Mol Physiol. 2004 Oct;287(4):L784-93. Epub 2004 May 28.


Rules of conduct for the cystic fibrosis anion channel.

Wine JJ.

Nat Med. 2003 Jul;9(7):827-8. No abstract available.


Absent secretion to vasoactive intestinal peptide in cystic fibrosis airway glands.

Joo NS, Irokawa T, Wu JV, Robbins RC, Whyte RI, Wine JJ.

J Biol Chem. 2002 Dec 27;277(52):50710-5. Epub 2002 Oct 3.


A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies.

Wagner JA, Nepomuceno IB, Messner AH, Moran ML, Batson EP, Dimiceli S, Brown BW, Desch JK, Norbash AM, Conrad CK, Guggino WB, Flotte TR, Wine JJ, Carter BJ, Reynolds TC, Moss RB, Gardner P.

Hum Gene Ther. 2002 Jul 20;13(11):1349-59.


Mucus secretion from single submucosal glands of pig. Stimulation by carbachol and vasoactive intestinal peptide.

Joo NS, Saenz Y, Krouse ME, Wine JJ.

J Biol Chem. 2002 Aug 2;277(31):28167-75. Epub 2002 May 13.


Natural animal models of human genetic diseases.

Wine JJ, Dean M, Glavac D.

Methods Mol Med. 2002;70:31-46. Review. No abstract available.


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