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Effect of ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis patients with G551D-CFTR.

Donaldson SH, Laube BL, Corcoran TE, Bhambhvani P, Zeman K, Ceppe A, Zeitlin PL, Mogayzel PJ Jr, Boyle M, Locke LW, Myerburg MM, Pilewski JM, Flanagan B, Rowe SM, Bennett WD.

JCI Insight. 2018 Dec 20;3(24). pii: 122695. doi: 10.1172/jci.insight.122695.


Benralizumab does not impair antibody response to seasonal influenza vaccination in adolescent and young adult patients with moderate to severe asthma: results from the Phase IIIb ALIZE trial.

Zeitlin PL, Leong M, Cole J, Mallory RM, Shih VH, Olsson RF, Goldman M; ALIZE study investigators.

J Asthma Allergy. 2018 Nov 20;11:181-192. doi: 10.2147/JAA.S172338. eCollection 2018.


Airway microbiota across age and disease spectrum in cystic fibrosis.

Zemanick ET, Wagner BD, Robertson CE, Ahrens RC, Chmiel JF, Clancy JP, Gibson RL, Harris WT, Kurland G, Laguna TA, McColley SA, McCoy K, Retsch-Bogart G, Sobush KT, Zeitlin PL, Stevens MJ, Accurso FJ, Sagel SD, Harris JK.

Eur Respir J. 2017 Nov 16;50(5). pii: 1700832. doi: 10.1183/13993003.00832-2017. Print 2017 Nov.


Pharmacokinetics and safety of cavosonstat (N91115) in healthy and cystic fibrosis adults homozygous for F508DEL-CFTR.

Donaldson SH, Solomon GM, Zeitlin PL, Flume PA, Casey A, McCoy K, Zemanick ET, Mandagere A, Troha JM, Shoemaker SA, Chmiel JF, Taylor-Cousar JL.

J Cyst Fibros. 2017 May;16(3):371-379. doi: 10.1016/j.jcf.2017.01.009. Epub 2017 Feb 13.


Digitoxin for Airway Inflammation in Cystic Fibrosis: Preliminary Assessment of Safety, Pharmacokinetics, and Dose Finding.

Zeitlin PL, Diener-West M, Callahan KA, Lee S, Talbot CC Jr, Pollard B, Boyle MP, Lechtzin N.

Ann Am Thorac Soc. 2017 Feb;14(2):220-229. doi: 10.1513/AnnalsATS.201608-649OC.


Clinical Phenotypes and Genotypic Spectrum of Cystic Fibrosis in Chinese Children.

Shen Y, Liu J, Zhong L, Mogayzel PJ Jr, Zeitlin PL, Sosnay PR, Zhao S.

J Pediatr. 2016 Apr;171:269-76.e1. doi: 10.1016/j.jpeds.2015.12.025. Epub 2016 Jan 27.


Direct interactions between ENaC gamma subunit and ClCN2 in cystic fibrosis epithelial cells.

Henry KR, Lee S, Walker D, Zeitlin PL.

Physiol Rep. 2015 Jan 27;3(1). pii: e12264. doi: 10.14814/phy2.12264. Print 2015 Jan 1.


Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Kerem E, Konstan MW, De Boeck K, Accurso FJ, Sermet-Gaudelus I, Wilschanski M, Elborn JS, Melotti P, Bronsveld I, Fajac I, Malfroot A, Rosenbluth DB, Walker PA, McColley SA, Knoop C, Quattrucci S, Rietschel E, Zeitlin PL, Barth J, Elfring GL, Welch EM, Branstrom A, Spiegel RJ, Peltz SW, Ajayi T, Rowe SM; Cystic Fibrosis Ataluren Study Group.

Lancet Respir Med. 2014 Jul;2(7):539-47. doi: 10.1016/S2213-2600(14)70100-6. Epub 2014 May 15.


Interference with ubiquitination in CFTR modifies stability of core glycosylated and cell surface pools.

Lee S, Henderson MJ, Schiffhauer E, Despanie J, Henry K, Kang PW, Walker D, McClure ML, Wilson L, Sorscher EJ, Zeitlin PL.

Mol Cell Biol. 2014 Jul;34(14):2554-65.


Dual activation of CFTR and CLCN2 by lubiprostone in murine nasal epithelia.

Schiffhauer ES, Vij N, Kovbasnjuk O, Kang PW, Walker D, Lee S, Zeitlin PL.

Am J Physiol Lung Cell Mol Physiol. 2013 Mar 1;304(5):L324-31. doi: 10.1152/ajplung.00277.2012. Epub 2013 Jan 11.


Antibody microarrays: analysis of cystic fibrosis.

Jozwik CE, Pollard HB, Srivastava M, Eidelman O, Fan Q, Darling TN, Zeitlin PL.

Methods Mol Biol. 2012;823:179-200. doi: 10.1007/978-1-60327-216-2_12.


N-acetylcysteine enhances cystic fibrosis sputum penetration and airway gene transfer by highly compacted DNA nanoparticles.

Suk JS, Boylan NJ, Trehan K, Tang BC, Schneider CS, Lin JM, Boyle MP, Zeitlin PL, Lai SK, Cooper MJ, Hanes J.

Mol Ther. 2011 Nov;19(11):1981-9. doi: 10.1038/mt.2011.160. Epub 2011 Aug 9.


Cystic fibrosis and sinusitis in children: outcomes and socioeconomic status.

Kovell LC, Wang J, Ishman SL, Zeitlin PL, Boss EF.

Otolaryngol Head Neck Surg. 2011 Jul;145(1):146-53. doi: 10.1177/0194599811400816.


Applications of proteomic technologies for understanding the premature proteolysis of CFTR.

Henderson MJ, Singh OV, Zeitlin PL.

Expert Rev Proteomics. 2010 Aug;7(4):473-86. doi: 10.1586/epr.10.42. Review.


Ubiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.

Henderson MJ, Vij N, Zeitlin PL.

J Biol Chem. 2010 Apr 9;285(15):11314-25. doi: 10.1074/jbc.M109.044057. Epub 2010 Feb 10.


Transient effectiveness of vitamin D2 therapy in pediatric cystic fibrosis patients.

Green DM, Leonard AR, Paranjape SM, Rosenstein BJ, Zeitlin PL, Mogayzel PJ Jr.

J Cyst Fibros. 2010 Mar;9(2):143-9. doi: 10.1016/j.jcf.2010.01.002. Epub 2010 Feb 8.


Description of a standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis.

Leonard A, Davis E, Rosenstein BJ, Zeitlin PL, Paranjape SM, Peeler D, Maynard C, Mogayzel PJ Jr.

J Pediatr Psychol. 2010 Jan-Feb;35(1):6-13. doi: 10.1093/jpepsy/jsp029. Epub 2009 May 6.


CFTR is a negative regulator of NFkappaB mediated innate immune response.

Vij N, Mazur S, Zeitlin PL.

PLoS One. 2009;4(2):e4664. doi: 10.1371/journal.pone.0004664. Epub 2009 Feb 27.


Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury.

Singh OV, Yaster M, Xu JT, Guan Y, Guan X, Dharmarajan AM, Raja SN, Zeitlin PL, Tao YX.

Proteomics. 2009 Mar;9(5):1241-53. doi: 10.1002/pmic.200800636.


The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles.

Suk JS, Lai SK, Wang YY, Ensign LM, Zeitlin PL, Boyle MP, Hanes J.

Biomaterials. 2009 May;30(13):2591-7. doi: 10.1016/j.biomaterials.2008.12.076. Epub 2009 Jan 26.


Cystic fibrosis and estrogens: a perfect storm.

Zeitlin PL.

J Clin Invest. 2008 Dec;118(12):3841-4. doi: 10.1172/JCI37778. Epub 2008 Nov 20.


Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.

Liang L, MacDonald K, Schwiebert EM, Zeitlin PL, Guggino WB.

Am J Physiol Cell Physiol. 2009 Jan;296(1):C131-41. doi: 10.1152/ajpcell.00346.2008. Epub 2008 Nov 5.


Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice.

MacDonald KD, McKenzie KR, Henderson MJ, Hawkins CE, Vij N, Zeitlin PL.

Am J Physiol Lung Cell Mol Physiol. 2008 Nov;295(5):L933-40. doi: 10.1152/ajplung.90221.2008. Epub 2008 Sep 19.


Cystic fibrosis presenting as recurrent pancreatitis in a young child with a normal sweat test and pancreas divisum: a case report.

Conklin L, Zeitlin PL, Cuffari C.

J Med Case Rep. 2008 May 23;2:176. doi: 10.1186/1752-1947-2-176.


Atypical cystic fibrosis and CFTR-related diseases.

Paranjape SM, Zeitlin PL.

Clin Rev Allergy Immunol. 2008 Dec;35(3):116-23. doi: 10.1007/s12016-008-8083-0. Review.


Dietary supplement use in pediatric patients with cystic fibrosis.

Murray KL, Lee CK, Mogayzel PJ Jr, Zeitlin PL, Rosenstein BJ.

Am J Health Syst Pharm. 2008 Mar 15;65(6):562-5. doi: 10.2146/ajhp070447.


Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells.

Singh OV, Pollard HB, Zeitlin PL.

Mol Cell Proteomics. 2008 Jun;7(6):1099-110. doi: 10.1074/mcp.M700303-MCP200. Epub 2008 Feb 19.


Protein microarray platforms for clinical proteomics.

Pollard HB, Srivastava M, Eidelman O, Jozwik C, Rothwell SW, Mueller GP, Jacobowitz DM, Darling T, Guggino WB, Wright J, Zeitlin PL, Paweletz CP.

Proteomics Clin Appl. 2007 Sep;1(9):934-52. doi: 10.1002/prca.200700154. Epub 2007 Aug 10.


CHOP transcription factor mediates IL-8 signaling in cystic fibrosis bronchial epithelial cells.

Vij N, Amoako MO, Mazur S, Zeitlin PL.

Am J Respir Cell Mol Biol. 2008 Feb;38(2):176-84. Epub 2007 Aug 20.


Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.

Moss RB, Milla C, Colombo J, Accurso F, Zeitlin PL, Clancy JP, Spencer LT, Pilewski J, Waltz DA, Dorkin HL, Ferkol T, Pian M, Ramsey B, Carter BJ, Martin DB, Heald AE.

Hum Gene Ther. 2007 Aug;18(8):726-32.


Patients with mutations in Gsalpha have reduced activation of a downstream target in epithelial tissues due to haploinsufficiency.

Hsu SC, Groman JD, Merlo CA, Naughton K, Zeitlin PL, Germain-Lee EL, Boyle MP, Cutting GR.

J Clin Endocrinol Metab. 2007 Oct;92(10):3941-8. Epub 2007 Jul 24.


Emerging drug treatments for cystic fibrosis.

Zeitlin PL.

Expert Opin Emerg Drugs. 2007 May;12(2):329-36. Review.


Levofloxacin pharmacokinetics in adult cystic fibrosis.

Lee CKK, Boyle MP, Diener-West M, Brass-Ernst L, Noschese M, Zeitlin PL.

Chest. 2007 Mar;131(3):796-802. doi: 10.1378/chest.06-1524.


Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation.

MacDonald KD, McKenzie KR, Zeitlin PL.

Paediatr Drugs. 2007;9(1):1-10. Review.


Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis.

Buranawuti K, Boyle MP, Cheng S, Steiner LL, McDougal K, Fallin MD, Merlo C, Zeitlin PL, Rosenstein BJ, Mogayzel PJ Jr, Wang X, Cutting GR.

J Med Genet. 2007 Mar;44(3):209-14. Epub 2006 Dec 11.


De novo biosynthetic profiling of high abundance proteins in cystic fibrosis lung epithelial cells.

Pollard HB, Eidelman O, Jozwik C, Huang W, Srivastava M, Ji XD, McGowan B, Norris CF, Todo T, Darling T, Mogayzel PJ, Zeitlin PL, Wright J, Guggino WB, Metcalf E, Driscoll WJ, Mueller G, Paweletz C, Jacobowitz DM.

Mol Cell Proteomics. 2006 Sep;5(9):1628-37. Epub 2006 Jul 7.


Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia.

Boyle MP, Enke RA, Reynolds JB, Mogayzel PJ Jr, Guggino WB, Zeitlin PL.

Virol J. 2006 Apr 22;3:29.


Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.

Wright JM, Merlo CA, Reynolds JB, Zeitlin PL, Garcia JG, Guggino WB, Boyle MP.

Am J Respir Cell Mol Biol. 2006 Sep;35(3):327-36. Epub 2006 Apr 13.


Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells.

Singh OV, Vij N, Mogayzel PJ Jr, Jozwik C, Pollard HB, Zeitlin PL.

J Proteome Res. 2006 Mar;5(3):562-71.


Regulation of the ClC-2 lung epithelial chloride channel by glycosylation of SP1.

Vij N, Zeitlin PL.

Am J Respir Cell Mol Biol. 2006 Jun;34(6):754-9. Epub 2006 Feb 2.


Serum proteomic signature for cystic fibrosis using an antibody microarray platform.

Srivastava M, Eidelman O, Jozwik C, Paweletz C, Huang W, Zeitlin PL, Pollard HB.

Mol Genet Metab. 2006 Apr;87(4):303-10. Epub 2006 Jan 10.


Acidic pH hyperpolarizes nasal potential difference.

Uwaifo O, Bamford P, Zeitlin PL, Blaisdell CJ.

Pediatr Pulmonol. 2006 Feb;41(2):151-7.


Positive expiratory pressure changes aerosol distribution in patients with cystic fibrosis.

Laube BL, Geller DE, Lin TC, Dalby RN, Diener-West M, Zeitlin PL.

Respir Care. 2005 Nov;50(11):1438-44.


Correlation between DNA transfer and cystic fibrosis airway epithelial cell correction after recombinant adeno-associated virus serotype 2 gene therapy.

Flotte TR, Schwiebert EM, Zeitlin PL, Carter BJ, Guggino WB.

Hum Gene Ther. 2005 Aug;16(8):921-8.


Down-regulation of IL-8 expression in human airway epithelial cells through helper-dependent adenoviral-mediated RNA interference.

Cao HB, Wang A, Martin B, Koehler DR, Zeitlin PL, Tanawell AK, Hu J.

Cell Res. 2005 Feb;15(2):111-9.


Safety and tolerability of denufosol tetrasodium inhalation solution, a novel P2Y2 receptor agonist: results of a phase 1/phase 2 multicenter study in mild to moderate cystic fibrosis.

Deterding R, Retsch-Bogart G, Milgram L, Gibson R, Daines C, Zeitlin PL, Milla C, Marshall B, Lavange L, Engels J, Mathews D, Gorden J, Schaberg A, Williams J, Ramsey B; Cystic Fibrosis Foundation Therapeutics Development Network.

Pediatr Pulmonol. 2005 Apr;39(4):339-48.


Evaluation of exposure and health care worker response to nebulized administration of tgAAVCF to patients with cystic fibrosis.

Croteau GA, Martin DB, Camp J, Yost M, Conrad C, Zeitlin PL, Heald AE.

Ann Occup Hyg. 2004 Nov;48(8):673-81. Epub 2004 Oct 26.


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