Antibody microarrays: analysis of cystic fibrosis.
Methods Mol. Biol., 823:179-200 (2012)
N-acetylcysteine enhances cystic fibrosis sputum penetration and airway gene transfer by highly compacted DNA nanoparticles.
Mol. Ther., Nov;19(11):1981-9 (2011)
Cystic fibrosis and sinusitis in children: outcomes and socioeconomic status.
Otolaryngol Head Neck Surg., Jul;145(1):146-53 (2011)
Applications of proteomic technologies for understanding the premature proteolysis of CFTR.
Expert Rev Proteomics., Aug;7(4):473-86 (2010)
Ubiquitin C-terminal hydrolase-L1 protects cystic fibrosis transmembrane conductance regulator from early stages of proteasomal degradation.
J. Biol. Chem., Apr;285(15):11314-25 (2010)
Transient effectiveness of vitamin D2 therapy in pediatric cystic fibrosis patients.
J. Cyst. Fibros., Mar;9(2):143-9 (2010)
Pseudomonas aeruginosa: can studies in engineered cells tell us why is it such a problem in people with cystic fibrosis? Focus on "Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas a
Am. J. Physiol., Cell Physiol., Aug;297(2):C235-7 (2009)
Description of a standardized nutrition classification plan and its relation to nutritional outcomes in children with cystic fibrosis.
J Pediatr Psychol., 35(1):6-13 (2010 Jan-Feb)
CFTR is a negative regulator of NFkappaB mediated innate immune response.
PLoS ONE., 4(2):e4664 (2009)
Proteome of synaptosome-associated proteins in spinal cord dorsal horn after peripheral nerve injury.
Proteomics., Mar;9(5):1241-53 (2009)
The penetration of fresh undiluted sputum expectorated by cystic fibrosis patients by non-adhesive polymer nanoparticles.
Biomaterials., May;30(13):2591-7 (2009)
Cystic fibrosis and estrogens: a perfect storm.
J. Clin. Invest., Dec;118(12):3841-4 (2008)
Spiperone, identified through compound screening, activates calcium-dependent chloride secretion in the airway.
Am. J. Physiol., Cell Physiol., Jan;296(1):C131-41 (2009)
Lubiprostone activates non-CFTR-dependent respiratory epithelial chloride secretion in cystic fibrosis mice.
Am. J. Physiol. Lung Cell Mol. Physiol., Nov;295(5):L933-40 (2008)
Cystic fibrosis presenting as recurrent pancreatitis in a young child with a normal sweat test and pancreas divisum: a case report.
J Med Case Rep., 2:176 (2008)
Atypical cystic fibrosis and CFTR-related diseases.
Clin Rev Allergy Immunol., Dec;35(3):116-23 (2008)
Dietary supplement use in pediatric patients with cystic fibrosis.
Am J Health Syst Pharm., Mar;65(6):562-5 (2008)
Chemical rescue of deltaF508-CFTR mimics genetic repair in cystic fibrosis bronchial epithelial cells.
Mol. Cell Proteomics., Jun;7(6):1099-110 (2008)
Protein microarray platforms for clinical proteomics.
Proteomics Clin Appl., Sep;1(9):934-52 (2007)
CHOP transcription factor mediates IL-8 signaling in cystic fibrosis bronchial epithelial cells.
Am. J. Respir. Cell Mol. Biol., Feb;38(2):176-84 (2008)
Repeated aerosolized AAV-CFTR for treatment of cystic fibrosis: a randomized placebo-controlled phase 2B trial.
Hum. Gene Ther., Aug;18(8):726-32 (2007)
Patients with mutations in Gsalpha have reduced activation of a downstream target in epithelial tissues due to haploinsufficiency.
J. Clin. Endocrinol. Metab., Oct;92(10):3941-8 (2007)
Emerging drug treatments for cystic fibrosis.
Expert Opin Emerg Drugs., May;12(2):329-36 (2007)
Levofloxacin pharmacokinetics in adult cystic fibrosis.
Chest., Mar;131(3):796-802 (2007)
Cystic fibrosis transmembrane regulator protein mutations: 'class' opportunity for novel drug innovation.
Paediatr Drugs., 9(1):1-10 (2007)
Variants in mannose-binding lectin and tumour necrosis factor alpha affect survival in cystic fibrosis.
J. Med. Genet., Mar;44(3):209-14 (2007)
Is it go or NO go for S-nitrosylation modification-based therapies of cystic fibrosis transmembrane regulator trafficking?
Mol. Pharmacol., Oct;70(4):1155-8 (2006)
De novo biosynthetic profiling of high abundance proteins in cystic fibrosis lung epithelial cells.
Mol. Cell Proteomics., Sep;5(9):1628-37 (2006)
Membrane-associated heparan sulfate is not required for rAAV-2 infection of human respiratory epithelia.
Virol. J., 3:29 (2006)
Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications.
J. Biol. Chem., Jun;281(25):17369-78 (2006)
Respiratory epithelial gene expression in patients with mild and severe cystic fibrosis lung disease.
Am. J. Respir. Cell Mol. Biol., Sep;35(3):327-36 (2006)
Pharmacoproteomics of 4-phenylbutyrate-treated IB3-1 cystic fibrosis bronchial epithelial cells.
J. Proteome Res., Mar;5(3):562-71 (2006)
Regulation of the ClC-2 lung epithelial chloride channel by glycosylation of SP1.
Am. J. Respir. Cell Mol. Biol., Jun;34(6):754-9 (2006)
Serum proteomic signature for cystic fibrosis using an antibody microarray platform.
Mol. Genet. Metab., Apr;87(4):303-10 (2006)
Acidic pH hyperpolarizes nasal potential difference.
Pediatr. Pulmonol., Feb;41(2):151-7 (2006)
Positive expiratory pressure changes aerosol distribution in patients with cystic fibrosis.
Respir Care., Nov;50(11):1438-44 (2005)
Correlation between DNA transfer and cystic fibrosis airway epithelial cell correction after recombinant adeno-associated virus serotype 2 gene therapy.
Hum. Gene Ther., Aug;16(8):921-8 (2005)
Down-regulation of IL-8 expression in human airway epithelial cells through helper-dependent adenoviral-mediated RNA interference.
Cell Res., Feb;15(2):111-9 (2005)
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.
Pediatr. Pulmonol., Apr;39(4):339-48 (2005)
Evaluation of exposure and health care worker response to nebulized administration of tgAAVCF to patients with cystic fibrosis.
Ann Occup Hyg., Nov;48(8):673-81 (2004)
Modulation of deltaF508 cystic fibrosis transmembrane regulator trafficking and function with 4-phenylbutyrate and flavonoids.
Am. J. Respir. Cell Mol. Biol., Sep;31(3):351-7 (2004)
Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials.
Pediatr. Pulmonol., May;37(5):385-92 (2004)
Repeated adeno-associated virus serotype 2 aerosol-mediated cystic fibrosis transmembrane regulator gene transfer to the lungs of patients with cystic fibrosis: a multicenter, double-blind, placebo-controlled trial.
Chest., Feb;125(2):509-21 (2004)
A phase I trial of intranasal Moli1901 for cystic fibrosis.
Chest., Jan;125(1):143-9 (2004)
Emerging drug treatments for cystic fibrosis.
Expert Opin Emerg Drugs., Nov;8(2):523-35 (2003)
Gene expression profile analysis of 4-phenylbutyrate treatment of IB3-1 bronchial epithelial cell line demonstrates a major influence on heat-shock proteins.
Physiol. Genomics., Jan;16(2):204-11 (2004)
Protein processing and degradation in pulmonary health and disease.
Am. J. Respir. Cell Mol. Biol., Nov;29(5):642-5 (2003)
Therapeutic strategies to correct malfunction of CFTR.
Paediatr Respir Rev., Jun;2(2):159-64 (2001)
Therapeutic approaches to repair defects in deltaF508 CFTR folding and cellular targeting.
Adv. Drug Deliv. Rev., Dec;54(11):1395-408 (2002)
ClC-5: ontogeny of an alternative chloride channel in respiratory epithelia.
Am. J. Physiol. Lung Cell Mol. Physiol., Mar;282(3):L501-7 (2002)
pH-regulated chloride secretion in fetal lung epithelia.
Am. J. Physiol. Lung Cell Mol. Physiol., Jun;278(6):L1248-55 (2000)
Sodium 4-phenylbutyrate downregulates Hsc70: implications for intracellular trafficking of DeltaF508-CFTR.
Am. J. Physiol., Cell Physiol., Feb;278(2):C259-67 (2000)
Perinatal regulation of the ClC-2 chloride channel in lung is mediated by Sp1 and Sp3.
Am. J. Physiol., Apr;276(4 Pt 1):L614-24 (1999)
Keratinocyte growth factor stimulates CLC-2 expression in primary fetal rat distal lung epithelial cells.
Am. J. Respir. Cell Mol. Biol., Apr;20(4):842-7 (1999)
Alternative mRNA splice variants of the rat ClC-2 chloride channel gene are expressed in lung: genomic sequence and organization of ClC-2.
Nucleic Acids Res., Oct;25(20):4153-9 (1997)
CFTR gene transduction in neonatal rabbits using an adeno-associated virus (AAV) vector.
Gene Ther., May;4(5):384-92 (1997)
Gestational and tissue-specific regulation of C1C-2 chloride channel expression.
Am. J. Physiol., Nov;271(5 Pt 1):L829-37 (1996)
A short CIC-2 mRNA transcript is produced by exon skipping.
Nucleic Acids Res., Sep;24(17):3453-7 (1996)
Prognosis in cystic fibrosis.
Curr Opin Pulm Med., Nov;1(6):444-9 (1995)
Alveolar stem cell transduction by an adeno-associated viral vector.
Gene Ther., Nov;2(9):623-31 (1995)
A1 receptor antagonist 8-cyclopentyl-1,3-dipropylxanthine selectively activates chloride efflux from human epithelial and mouse fibroblast cell lines expressing the cystic fibrosis transmembrane regulator delta F508 mutation.
Biochemistry., Jul;34(28):9079-87 (1995)
CIC-2: a developmentally dependent chloride channel expressed in the fetal lung and downregulated after birth.
Am. J. Respir. Cell Mol. Biol., Jun;12(6):597-604 (1995)
Adeno-associated virus vector gene expression occurs in nondividing cells in the absence of vector DNA integration.
Am. J. Respir. Cell Mol. Biol., Nov;11(5):517-21 (1994)
Stable in vivo expression of the cystic fibrosis transmembrane conductance regulator with an adeno-associated virus vector.
Proc. Natl. Acad. Sci. U.S.A., Nov;90(22):10613-7 (1993)
Cystic fibrosis gene and protein expression during fetal lung development.
Am. J. Respir. Cell Mol. Biol., Feb;8(2):201-8 (1993)
Gene expression from adeno-associated virus vectors in airway epithelial cells.
Am. J. Respir. Cell Mol. Biol., Sep;7(3):349-56 (1992)
Effect of hypoxia on endothelin-1 production by pulmonary vascular endothelial cells.
Biochim. Biophys. Acta., Mar;1134(2):105-11 (1992)
Severe deficiency of cystic fibrosis transmembrane conductance regulator messenger RNA carrying nonsense mutations R553X and W1316X in respiratory epithelial cells of patients with cystic fibrosis.
J. Clin. Invest., Dec;88(6):1880-5 (1991)
Immunocytochemical localization of the cystic fibrosis gene product CFTR.
Proc. Natl. Acad. Sci. U.S.A., Oct;88(20):9262-6 (1991)
Down-regulation of cystic fibrosis gene mRNA transcript levels and induction of the cystic fibrosis chloride secretory phenotype in epithelial cells by phorbol ester.
J. Biol. Chem., Jun;266(16):10319-23 (1991)
Gene transfer by lipofection in rabbit and human secretory epithelial cells.
Pflugers Arch., Nov;415(2):198-203 (1989)