Airway delivery of low-dose miglustat normalizes nasal potential difference in F508del cystic fibrosis mice.

PubWeight™: 0.84‹?›

🔗 View Article (PMID 19299496)

Published in Am J Respir Crit Care Med on March 19, 2009

Authors

Bob Lubamba1, Jean Lebacq, Patrick Lebecque, Rita Vanbever, Anissa Leonard, Pierre Wallemacq, Teresinha Leal

Author Affiliations

1: Department of Clinical Chemistry, Université Catholique de Louvain, Brussels, Belgium.

Associated clinical trials:

Does a Nasal Instillation of Miglustat Normalize the Nasal Potential Difference in Cystic Fibrosis Patients ? | NCT00945347

Articles by these authors

Reference ranges for spirometry across all ages: a new approach. Am J Respir Crit Care Med (2007) 4.03

Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation. Thorax (2011) 2.84

Associations between cytomegalovirus infection and functional impairment and frailty in the BELFRAIL Cohort. J Am Geriatr Soc (2011) 2.38

Preclinical evidence that sildenafil and vardenafil activate chloride transport in cystic fibrosis. Am J Respir Crit Care Med (2007) 2.18

Insufficient β-lactam concentrations in the early phase of severe sepsis and septic shock. Crit Care (2010) 2.11

The effect of CYP3A5 and MDR1 (ABCB1) polymorphisms on cyclosporine and tacrolimus dose requirements and trough blood levels in stable renal transplant patients. Pharmacogenetics (2004) 2.06

A randomized placebo-controlled trial of miglustat in cystic fibrosis based on nasal potential difference. J Cyst Fibros (2012) 2.05

Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease. Hum Mutat (2009) 1.71

Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis. Am J Respir Crit Care Med (2010) 1.71

The European Register of Specialists in Clinical Chemistry and Laboratory Medicine: guide to the Register, version 3-2010. Clin Chem Lab Med (2010) 1.68

Mutations of the cystic fibrosis gene and intermediate sweat chloride levels in children. Am J Respir Crit Care Med (2002) 1.68

Skin electroporation for transdermal and topical delivery. Adv Drug Deliv Rev (2004) 1.48

The impact of the assay for measuring albumin on corrected ('adjusted') calcium concentrations. Nephrol Dial Transplant (2009) 1.43

Prolonged unconsciousness in a patient on automated peritoneal dialysis. Eur J Emerg Med (2006) 1.38

Congenital tracheal malformation in cystic fibrosis transmembrane conductance regulator-deficient mice. J Physiol (2008) 1.30

Comparison of spirometric reference values. Pediatr Pulmonol (2004) 1.28

Effect of a new functional CYP3A4 polymorphism on calcineurin inhibitors' dose requirements and trough blood levels in stable renal transplant patients. Pharmacogenomics (2011) 1.27

Revisiting the loading dose of amikacin for patients with severe sepsis and septic shock. Crit Care (2010) 1.26

Sirolimus and tacrolimus trough concentrations and dose requirements after kidney transplantation in relation to CYP3A5 and MDR1 polymorphisms and steroids. Transplantation (2005) 1.21

SUMOylation of tissue transglutaminase as link between oxidative stress and inflammation. J Immunol (2009) 1.14

Azithromycin reduces spontaneous and induced inflammation in DeltaF508 cystic fibrosis mice. Respir Res (2006) 1.12

Potentiation of cyclophosphamide chemotherapy using the anti-angiogenic drug thalidomide: importance of optimal scheduling to exploit the 'normalization' window of the tumor vasculature. Cancer Lett (2006) 1.11

Role of TRPC1 channel in skeletal muscle function. Am J Physiol Cell Physiol (2009) 1.11

Azithromycin reduces exaggerated cytokine production by M1 alveolar macrophages in cystic fibrosis. Am J Respir Cell Mol Biol (2009) 1.11

Functional role of store-operated and stretch-activated channels in murine adult skeletal muscle fibres. J Physiol (2006) 1.09

Comparison of calibrated chromogenic anti-Xa assay and PT tests with LC-MS/MS for the therapeutic monitoring of patients treated with rivaroxaban. Thromb Haemost (2013) 1.02

Does in utero exposure to heavy maternal smoking induce nicotine withdrawal symptoms in neonates? Pediatr Res (2004) 1.02

Cystic fibrosis is associated with a defect in apical receptor-mediated endocytosis in mouse and human kidney. J Am Soc Nephrol (2007) 1.02

The BELFRAIL (BFC80+) study: a population-based prospective cohort study of the very elderly in Belgium. BMC Geriatr (2010) 1.01

Basic protocol for transepithelial nasal potential difference measurements. J Cyst Fibros (2004) 1.01

Validation of an ELISA-based screening assay for the detection of amphetamine, MDMA and MDA in blood and oral fluid. Forensic Sci Int (2005) 1.01

Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy. Clin Biochem (2012) 1.01

The EC4 European syllabus for post-graduate training in clinical chemistry and laboratory medicine: version 4--2012. Clin Chem Lab Med (2012) 0.99

The particle has landed--characterizing the fate of inhaled pharmaceuticals. J Aerosol Med Pulm Drug Deliv (2010) 0.97

The delivery site of a monovalent influenza vaccine within the respiratory tract impacts on the immune response. Immunology (2007) 0.96

Aerosolization properties, surface composition and physical state of spray-dried protein powders. J Control Release (2004) 0.96

Influence of host genetic factors on efavirenz plasma and intracellular pharmacokinetics in HIV-1-infected patients. Pharmacogenomics (2010) 0.95

Essential role of TRPV2 ion channel in the sensitivity of dystrophic muscle to eccentric contractions. FEBS Lett (2009) 0.94

Limited sampling models and Bayesian estimation for mycophenolic acid area under the curve prediction in stable renal transplant patients co-medicated with ciclosporin or sirolimus. Clin Pharmacokinet (2009) 0.94

A simultaneous d-optimal designed study for population pharmacokinetic analyses of mycophenolic Acid and tacrolimus early after renal transplantation. J Clin Pharmacol (2011) 0.93

Vancomycin penetration of uninfected pleural fluid exudate after continuous or intermittent infusion. Antimicrob Agents Chemother (2003) 0.93

Role of IGF-I and IGFBPs in the changes of mass and phenotype induced in rat soleus muscle by clenbuterol. Am J Physiol Endocrinol Metab (2002) 0.93

An overview of monitoring and supplementation of omega 3 fatty acids in cystic fibrosis. Clin Biochem (2007) 0.93

Transient elastography in patients with cystic fibrosis. Pediatr Radiol (2010) 0.92

1199G>A and 2677G>T/A polymorphisms of ABCB1 independently affect tacrolimus concentration in hepatic tissue after liver transplantation. Pharmacogenet Genomics (2007) 0.92

Time of drug administration, CYP3A5 and ABCB1 genotypes, and analytical method influence tacrolimus pharmacokinetics: a population pharmacokinetic study. Ther Drug Monit (2009) 0.91

Omalizumab for treatment of ABPA exacerbations in CF patients. Pediatr Pulmonol (2009) 0.91

The role of epithelial P2Y2 and P2Y4 receptors in the regulation of intestinal chloride secretion. Br J Pharmacol (2005) 0.91

Iodine and iron status of pregnant women in Lubumbashi, Democratic Republic of Congo. Public Health Nutr (2013) 0.90

CYP3A5 and ABCB1 polymorphisms influence tacrolimus concentrations in peripheral blood mononuclear cells after renal transplantation. Pharmacogenomics (2010) 0.90

Hyperhomocysteinemia and venous thromboembolism: a risk factor more prevalent in the elderly and in idiopathic cases. Thromb Res (2002) 0.90

Pharmacokinetics of a loading dose of amikacin in septic patients undergoing continuous renal replacement therapy. Int J Antimicrob Agents (2011) 0.89

Glycosomal ABC transporters of Trypanosoma brucei: characterisation of their expression, topology and substrate specificity. Int J Parasitol (2010) 0.89

Pulmonary delivery of growth hormone using dry powders and visualization of its local fate in rats. J Control Release (2004) 0.88

The glomerular filtration rate estimated by new and old equations as a predictor of important outcomes in elderly patients. BMC Med (2014) 0.88

The future of hospital laboratories. Position statement from the Royal Belgian Society of Clinical Chemistry (RBSCC). Clin Chem Lab Med (2009) 0.88

Significance of serum immune markers in identification of global functional impairment in the oldest old: cross-sectional results from the BELFRAIL study. Age (Dordr) (2013) 0.87

Sirtuin inhibition attenuates the production of inflammatory cytokines in lipopolysaccharide-stimulated macrophages. Biochem Biophys Res Commun (2012) 0.87

Preclinical models for pulmonary drug delivery. Expert Opin Drug Deliv (2009) 0.87

The prevalence of cardiac dysfunction and the correlation with poor functioning among the very elderly. Int J Cardiol (2011) 0.87

Comparison of culture and qPCR for the detection of Pseudomonas aeruginosa in not chronically infected cystic fibrosis patients. BMC Microbiol (2010) 0.87

Liquid chromatography-tandem mass spectrometry or automated immunoassays: what are the future trends in therapeutic drug monitoring? Clin Chem (2012) 0.87

Impact of CYP3A4*22 allele on tacrolimus pharmacokinetics in early period after renal transplantation: toward updated genotype-based dosage guidelines. Ther Drug Monit (2013) 0.87

Correlation of tacrolimus levels in peripheral blood mononuclear cells with histological staging of rejection after liver transplantation: preliminary results of a prospective study. Transpl Int (2011) 0.87

Barcelona Consensus on Biomarker-Based Immunosuppressive Drugs Management in Solid Organ Transplantation. Ther Drug Monit (2016) 0.86

Immunomodulatory activity of vardenafil on induced lung inflammation in cystic fibrosis mice. J Cyst Fibros (2012) 0.86

Performance of serum cystatin-C versus serum creatinine in subjects with type 1 diabetes. Diabetes Care (2003) 0.85

Monitoring of urea and potassium by reverse iontophoresis in vitro. Pharm Res (2007) 0.85

Systemic delivery of parathyroid hormone (1-34) using inhalation dry powders in rats. J Pharm Sci (2003) 0.85

Dysregulated proinflammatory and fibrogenic phenotype of fibroblasts in cystic fibrosis. PLoS One (2013) 0.85

Automated serum protein electrophoresis by Capillarys. Clin Chem Lab Med (2003) 0.84

Glucose homeostasis and genotype-phenotype interplay in cystic fibrosis patients with CFTR gene deltaF508 mutation. Diabetes Care (2007) 0.84

Azithromycin fails to reduce increased expression of neutrophil-related cytokines in primary-cultured epithelial cells from cystic fibrosis mice. J Cyst Fibros (2009) 0.84

Endoxifen levels and its association with CYP2D6 genotype and phenotype: evaluation of a southern Brazilian population under tamoxifen pharmacotherapy. Ther Drug Monit (2012) 0.84

The influence of genetic polymorphisms of cytochrome P450 3A5 and ABCB1 on starting dose- and weight-standardized tacrolimus trough concentrations after kidney transplantation in relation to renal function. Clin Chem Lab Med (2006) 0.84