Published in J Cyst Fibros on August 01, 2004
Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa. Am J Physiol Cell Physiol (2009) 1.72
Detection of cystic fibrosis transmembrane conductance regulator activity in early-phase clinical trials. Proc Am Thorac Soc (2007) 1.62
Characteristics of Cl- uptake in rat alveolar type I cells. Am J Physiol Lung Cell Mol Physiol (2009) 1.56
Genetically encoded optical sensors for monitoring of intracellular chloride and chloride-selective channel activity. Front Mol Neurosci (2009) 1.25
Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation. Autophagy (2014) 1.21
Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels. J Biol Chem (2012) 0.92
FKBP38 peptidylprolyl isomerase promotes the folding of cystic fibrosis transmembrane conductance regulator in the endoplasmic reticulum. J Biol Chem (2011) 0.86
A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR. Cell Death Differ (2016) 0.83
Sensitivity of chloride efflux vs. transepithelial measurements in mixed CF and normal airway epithelial cell populations. Cell Physiol Biochem (2011) 0.81
Time resolved secretion of chloride from a monolayer of mucin-secreting epithelial cells. Eur Biophys J (2007) 0.79
Thymosin α1 represents a potential potent single-molecule-based therapy for cystic fibrosis. Nat Med (2017) 0.75
Lifetime risk of symptomatic knee osteoarthritis. Arthritis Rheum (2008) 4.50
Association of warfarin therapy duration after bioprosthetic aortic valve replacement with risk of mortality, thromboembolic complications, and bleeding. JAMA (2012) 2.85
Confocal light absorption and scattering spectroscopic microscopy monitors organelles in live cells with no exogenous labels. Proc Natl Acad Sci U S A (2007) 2.56
Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax (2013) 2.42
Association of heart failure severity with risk of diabetes: a Danish nationwide cohort study. Diabetologia (2014) 2.08
Quality, size, and composition of pediatric endobronchial biopsies in cystic fibrosis. Chest (2007) 2.05
Efficacy of post-operative clopidogrel treatment in patients revascularized with coronary artery bypass grafting after myocardial infarction. J Am Coll Cardiol (2011) 2.04
In vitro prediction of stop-codon suppression by intravenous gentamicin in patients with cystic fibrosis: a pilot study. BMC Med (2007) 1.82
Cystic fibrosis. BMJ (2007) 1.82
CpG-free plasmids confer reduced inflammation and sustained pulmonary gene expression. Nat Biotechnol (2008) 1.73
Effects of epilepsy and selected antiepileptic drugs on risk of myocardial infarction, stroke, and death in patients with or without previous stroke: a nationwide cohort study. Pharmacoepidemiol Drug Saf (2011) 1.71
Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease. Hum Mutat (2009) 1.71
Airway remodelling and its relationship to inflammation in cystic fibrosis. Thorax (2010) 1.67
Normal function of the cystic fibrosis conductance regulator protein can be associated with homozygous (Delta)F508 mutation. Pediatr Res (2002) 1.61
Association between Hsp90 and the ClC-2 chloride channel upregulates channel function. Am J Physiol Cell Physiol (2005) 1.56
Beta-defensin genomic copy number is not a modifier locus for cystic fibrosis. J Negat Results Biomed (2005) 1.55
Dancing to our own tune: understandings of advanced nursing practice in British Columbia. Nurs Leadersh (Tor Ont) (2004) 1.52
NH3 is involved in the NH4+ transport induced by the functional expression of the human Rh C glycoprotein. J Biol Chem (2004) 1.51
Plasma phospholipid fatty acids are influenced by a ketogenic diet enriched with n-3 fatty acids in children with epilepsy. Epilepsy Res (2006) 1.51
Evaluation of MRP1-5 gene expression in cystic fibrosis patients homozygous for the delta F508 mutation. Pediatr Res (2003) 1.50
Distribution of ClC-2 chloride channel in rat and human epithelial tissues. Am J Physiol Cell Physiol (2002) 1.44
Transfection efficiency and toxicity following delivery of naked plasmid DNA and cationic lipid-DNA complexes to ovine lung segments. Mol Ther (2003) 1.43
Risk of stroke after coronary artery bypass grafting: effect of age and comorbidities. Stroke (2011) 1.43
Association of hypoglycemic treatment regimens with cardiovascular outcomes in overweight and obese subjects with type 2 diabetes: a substudy of the SCOUT trial. Diabetes Care (2013) 1.41
Sulfonylurea in combination with insulin is associated with increased mortality compared with a combination of insulin and metformin in a retrospective Danish nationwide study. Diabetologia (2014) 1.40
Airway remodelling in children with cystic fibrosis. Thorax (2007) 1.38
Omega-3 fatty acid supplementation prevents hepatic steatosis in a murine model of nonalcoholic fatty liver disease. Pediatr Res (2005) 1.33
Increased airway smooth muscle mass in children with asthma, cystic fibrosis, and non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med (2008) 1.27
Gene transfer to the lung: lessons learned from more than 2 decades of CF gene therapy. Adv Drug Deliv Rev (2008) 1.25
Confocal light absorption and scattering spectroscopic microscopy. Appl Opt (2007) 1.18
Quantitative trait loci (QTL) for lean body mass and body length in MRL/MPJ and SJL/J F(2) mice. Funct Integr Genomics (2002) 1.18
Direct visualization of Propionibacterium acnes in prostate tissue by multicolor fluorescent in situ hybridization assay. J Clin Microbiol (2007) 1.18
Decreased expression of peroxisome proliferator activated receptor gamma in cftr-/- mice. J Cell Physiol (2004) 1.17
Barriers to and new approaches for gene therapy and gene delivery in cystic fibrosis. Adv Drug Deliv Rev (2002) 1.17
Chimeric constructs endow the human CFTR Cl- channel with the gating behavior of murine CFTR. Proc Natl Acad Sci U S A (2007) 1.17
Antibodies for CFTR studies. J Cyst Fibros (2004) 1.06
Critical appraisal of the mouse model of myocardial infarction. Exp Physiol (2004) 1.05
Discovery of novel potent ΔF508-CFTR correctors that target the nucleotide binding domain. EMBO Mol Med (2013) 1.04
Lung clearance index and high-resolution computed tomography scores in primary ciliary dyskinesia. Am J Respir Crit Care Med (2013) 1.04
Effects of oral glucose-lowering drugs on long term outcomes in patients with diabetes mellitus following myocardial infarction not treated with emergent percutaneous coronary intervention--a retrospective nationwide cohort study. Cardiovasc Diabetol (2010) 1.04
Rescue of DeltaF508-CFTR (cystic fibrosis transmembrane conductance regulator) by curcumin: involvement of the keratin 18 network. J Pharmacol Exp Ther (2006) 1.04
Toward gene therapy for cystic fibrosis using a lentivirus pseudotyped with Sendai virus envelopes. Mol Ther (2010) 1.03
Inefficient cationic lipid-mediated siRNA and antisense oligonucleotide transfer to airway epithelial cells in vivo. Respir Res (2006) 1.03
Neutrophil elastase degrades cystic fibrosis transmembrane conductance regulator via calpains and disables channel function in vitro and in vivo. Am J Respir Crit Care Med (2012) 1.03
Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes. Am J Respir Crit Care Med (2005) 1.03
Clinical characteristics and major comorbidities in heart failure patients more than 85 years of age compared with younger age groups. Eur J Heart Fail (2011) 1.02
Basic protocol for transepithelial nasal potential difference measurements. J Cyst Fibros (2004) 1.01
Down-regulation of the anti-inflammatory protein annexin A1 in cystic fibrosis knock-out mice and patients. Mol Cell Proteomics (2005) 1.00
Cystic fibrosis enters the proteomics scene: new answers to old questions. Proteomics (2006) 1.00
Biochemical methods to assess CFTR expression and membrane localization. J Cyst Fibros (2004) 1.00
Cystic fibrosis transmembrane regulator inhibitors CFTR(inh)-172 and GlyH-101 target mitochondrial functions, independently of chloride channel inhibition. J Pharmacol Exp Ther (2010) 0.99
Expression of the human erythroid Rh glycoprotein (RhAG) enhances both NH3 and NH4+ transport in HeLa cells. Pflugers Arch (2005) 0.99
Enhanced lung gene expression after aerosol delivery of concentrated pDNA/PEI complexes. Mol Ther (2008) 0.99
Biomarkers for cystic fibrosis lung disease: application of SELDI-TOF mass spectrometry to BAL fluid. J Cyst Fibros (2008) 0.98
Airway gene therapy. Adv Genet (2005) 0.98
Inhibition of matrix metalloproteinases increases PPAR-alpha and IL-6 and prevents dietary-induced hepatic steatosis and injury in a murine model. Am J Physiol Gastrointest Liver Physiol (2006) 0.97
Potential difference measurements in the lower airway of children with and without cystic fibrosis. Am J Respir Crit Care Med (2005) 0.97
Peroxiredoxin 6 fails to limit phospholipid peroxidation in lung from Cftr-knockout mice subjected to oxidative challenge. PLoS One (2009) 0.97
Uric acid as a risk factor for cardiovascular disease and mortality in overweight/obese individuals. PLoS One (2013) 0.97
Aerosol delivery of DNA/liposomes to the lung for cystic fibrosis gene therapy. Hum Gene Ther Clin Dev (2014) 0.97
Valproate attenuates the risk of myocardial infarction in patients with epilepsy: a nationwide cohort study. Pharmacoepidemiol Drug Saf (2010) 0.97
Distinct patterns of inflammation in the airway lumen and bronchial mucosa of children with cystic fibrosis. Thorax (2011) 0.96
Cell-specific posttranscriptional regulation of CFTR gene expression via influence of MAPK cascades on 3'UTR part of transcripts. Am J Physiol Cell Physiol (2005) 0.96
Omega-3 fatty acids improve hepatic steatosis in a murine model: potential implications for the marginal steatotic liver donor. Transplantation (2005) 0.96
Blue native/SDS-PAGE analysis reveals reduced expression of the mClCA3 protein in cystic fibrosis knock-out mice. Mol Cell Proteomics (2005) 0.95
TGF-beta 1 downregulates CFTR expression and function in nasal polyps of non-CF patients. Am J Physiol Lung Cell Mol Physiol (2004) 0.94
Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein. Biochem Biophys Res Commun (2003) 0.94
Identification of protein cofactors necessary for sequence-specific plasmid DNA nuclear import. Mol Ther (2009) 0.94
Systematic review of progesterone for the prevention of preterm birth in singleton pregnancies. Acta Obstet Gynecol Scand (2009) 0.94
The efficacy of a 'master switch gene' HIF-1alpha in a porcine model of chronic myocardial ischaemia. Eur Heart J (2005) 0.94
Nasal polyps in cystic fibrosis: clinical endoscopic study with nasal lavage fluid analysis. Chest (2002) 0.94
Effects of hyperosmotic stress on cultured airway epithelial cells. Cell Tissue Res (2007) 0.93
Moving forward: cystic fibrosis gene therapy. Hum Mol Genet (2013) 0.93
A neutral variant involved in a complex CFTR allele contributes to a severe cystic fibrosis phenotype. Hum Genet (2005) 0.93
Coronin-1 is associated with neutrophil survival and is cleaved during apoptosis: potential implication in neutrophils from cystic fibrosis patients. J Immunol (2009) 0.93
A mechanism accounting for the low cellular level of linoleic acid in cystic fibrosis and its reversal by DHA. J Lipid Res (2008) 0.93
Lipid mapping of colonic mucosa by cluster TOF-SIMS imaging and multivariate analysis in cftr knockout mice. J Lipid Res (2010) 0.92
Secreted Gaussia luciferase as a sensitive reporter gene for in vivo and ex vivo studies of airway gene transfer. Biomaterials (2011) 0.92
Effect of duramycin on chloride transport and intracellular calcium concentration in cystic fibrosis and non-cystic fibrosis epithelia. APMIS (2010) 0.91
S-Nitrosoglutathione induces functional DeltaF508-CFTR in airway epithelial cells. Biochem Biophys Res Commun (2002) 0.91
Gene complementation of airway epithelium in the cystic fibrosis mouse is necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities. Hum Mol Genet (2002) 0.91
Curcumin does not stimulate cAMP-mediated chloride transport in cystic fibrosis airway epithelial cells. Biochem Biophys Res Commun (2004) 0.91
Progress in gene and cell therapy for cystic fibrosis lung disease. Curr Pharm Des (2012) 0.91
Anemia in the general population: prevalence, clinical correlates and prognostic impact. Eur J Epidemiol (2014) 0.90
Gender differences in the Scandinavian cystic fibrosis population. Pediatr Pulmonol (2010) 0.90
Evidence of increased flux to n-6 docosapentaenoic acid in phospholipids of pancreas from cftr-/- knockout mice. Metabolism (2006) 0.90
A novel lipidomic strategy reveals plasma phospholipid signatures associated with respiratory disease severity in cystic fibrosis patients. PLoS One (2009) 0.90
CFTR and tight junctions in cultured bronchial epithelial cells. Exp Mol Pathol (2009) 0.90
Emerging significance of plasmid DNA nuclear import in gene therapy. Adv Drug Deliv Rev (2003) 0.90
Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients. J Lipid Res (2011) 0.90
Global proteomic approach unmasks involvement of keratins 8 and 18 in the delivery of cystic fibrosis transmembrane conductance regulator (CFTR)/deltaF508-CFTR to the plasma membrane. Proteomics (2004) 0.90
Diagnostic significance of measurements of specific IgG antibodies to Pseudomonas aeruginosa by three different serological methods. J Cyst Fibros (2008) 0.90
Activation of CFTR by genistein in human airway epithelial cell lines. Biochem Biophys Res Commun (2003) 0.90