Published in Biochim Biophys Acta on February 13, 2006
Measurements of CFTR-mediated Cl- secretion in human rectal biopsies constitute a robust biomarker for Cystic Fibrosis diagnosis and prognosis. PLoS One (2012) 0.92
Syntaxin 3 is necessary for cAMP- and cGMP-regulated exocytosis of CFTR: implications for enterotoxigenic diarrhea. Am J Physiol Cell Physiol (2010) 0.87
Ceramide accumulation mediates inflammation, cell death and infection susceptibility in cystic fibrosis. Nat Med (2008) 3.41
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
Population structure of Pseudomonas aeruginosa. Proc Natl Acad Sci U S A (2007) 2.56
Activities of Pseudomonas aeruginosa effectors secreted by the Type III secretion system in vitro and during infection. Infect Immun (2005) 2.51
Highly adherent small-colony variants of Pseudomonas aeruginosa in cystic fibrosis lung infection. J Med Microbiol (2003) 2.17
Gene islands integrated into tRNA(Gly) genes confer genome diversity on a Pseudomonas aeruginosa clone. J Bacteriol (2002) 2.14
Quorum sensing: the power of cooperation in the world of Pseudomonas. Environ Microbiol (2005) 2.12
Pseudomonas aeruginosa microevolution during cystic fibrosis lung infection establishes clones with adapted virulence. Am J Respir Crit Care Med (2009) 2.08
Inhalation treatment with glutathione in patients with cystic fibrosis. A randomized clinical trial. Am J Respir Crit Care Med (2013) 2.07
Sequence analysis of the mobile genome island pKLC102 of Pseudomonas aeruginosa C. J Bacteriol (2004) 2.04
The neglected intrinsic resistome of bacterial pathogens. PLoS One (2008) 1.82
European best practice guidelines for cystic fibrosis neonatal screening. J Cyst Fibros (2009) 1.75
A cystic fibrosis epidemic strain of Pseudomonas aeruginosa displays enhanced virulence and antimicrobial resistance. J Bacteriol (2005) 1.74
Genome diversity of Pseudomonas aeruginosa PAO1 laboratory strains. J Bacteriol (2009) 1.73
Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease. Hum Mutat (2009) 1.71
Microevolution of the major common Pseudomonas aeruginosa clones C and PA14 in cystic fibrosis lungs. Environ Microbiol (2011) 1.70
Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data. Thorax (2010) 1.61
Requirement for galectin-3 in apical protein sorting. Curr Biol (2006) 1.55
Impaired trafficking of mutants of lysosomal glucocerebrosidase in Gaucher's disease. Int J Biochem Cell Biol (2005) 1.50
Pseudomonas aeruginosa Genomic Structure and Diversity. Front Microbiol (2011) 1.43
Diversity of the abundant pKLC102/PAGI-2 family of genomic islands in Pseudomonas aeruginosa. J Bacteriol (2006) 1.43
Expression analysis of a highly adherent and cytotoxic small colony variant of Pseudomonas aeruginosa isolated from a lung of a patient with cystic fibrosis. J Bacteriol (2004) 1.43
Deglycosylation by small intestinal epithelial cell beta-glucosidases is a critical step in the absorption and metabolism of dietary flavonoid glycosides in humans. Eur J Nutr (2003) 1.40
The crystal structure of SdsA1, an alkylsulfatase from Pseudomonas aeruginosa, defines a third class of sulfatases. Proc Natl Acad Sci U S A (2006) 1.39
Global features of the Pseudomonas putida KT2440 genome sequence. Environ Microbiol (2002) 1.39
Global features of sequences of bacterial chromosomes, plasmids and phages revealed by analysis of oligonucleotide usage patterns. BMC Bioinformatics (2004) 1.34
Endocytotic segregation of gliadin peptide 31-49 in enterocytes. Gut (2009) 1.34
Global regulation of quorum sensing and virulence by VqsR in Pseudomonas aeruginosa. Microbiology (2004) 1.32
Alteration of the pulmonary surfactant system in full-term infants with hereditary ABCA3 deficiency. Am J Respir Crit Care Med (2006) 1.30
Genes that determine immunology and inflammation modify the basic defect of impaired ion conductance in cystic fibrosis epithelia. J Med Genet (2010) 1.26
Structure of Pseudomonas aeruginosa populations analyzed by single nucleotide polymorphism and pulsed-field gel electrophoresis genotyping. J Bacteriol (2004) 1.24
Effect of treatment with dornase alpha on airway inflammation in patients with cystic fibrosis. Am J Respir Crit Care Med (2003) 1.24
Structural basis for substrate selectivity in human maltase-glucoamylase and sucrase-isomaltase N-terminal domains. J Biol Chem (2010) 1.24
Both cleavage products of the mCLCA3 protein are secreted soluble proteins. J Biol Chem (2006) 1.22
Sequence diversity of the mucABD locus in Pseudomonas aeruginosa isolates from patients with cystic fibrosis. Microbiology (2006) 1.20
Biological cost of hypermutation in Pseudomonas aeruginosa strains from patients with cystic fibrosis. Microbiology (2007) 1.20
Pseudomonas aeruginosa and Burkholderia cepacia in cystic fibrosis: genome evolution, interactions and adaptation. Int J Med Microbiol (2004) 1.18
Caenorhabditis elegans semi-automated liquid screen reveals a specialized role for the chemotaxis gene cheB2 in Pseudomonas aeruginosa virulence. PLoS Pathog (2009) 1.15
The CLCA gene locus as a modulator of the gastrointestinal basic defect in cystic fibrosis. Hum Genet (2004) 1.14
The genome structure of Pseudomonas putida: high-resolution mapping and microarray analysis. Environ Microbiol (2002) 1.13
Think big--giant genes in bacteria. Environ Microbiol (2008) 1.13
Origins of cystic fibrosis lung disease. N Engl J Med (2015) 1.12
Genome-wide transcriptional profiling of the steady-state response of Pseudomonas aeruginosa to hydrogen peroxide. J Bacteriol (2005) 1.10
Functional genomics of stress response in Pseudomonas putida KT2440. J Bacteriol (2006) 1.09
Differentiation of regions with atypical oligonucleotide composition in bacterial genomes. BMC Bioinformatics (2005) 1.08
Compound heterozygous mutations affect protein folding and function in patients with congenital sucrase-isomaltase deficiency. Gastroenterology (2008) 1.07
Inter- and intraclonal diversity of the Pseudomonas aeruginosa proteome manifests within the secretome. J Bacteriol (2003) 1.07
Characterization of poxB, a chromosomal-encoded Pseudomonas aeruginosa oxacillinase. Gene (2005) 1.06
The prostate specific membrane antigen regulates the expression of IL-6 and CCL5 in prostate tumour cells by activating the MAPK pathways. PLoS One (2009) 1.06
Genes in the vicinity of CFTR modulate the cystic fibrosis phenotype in highly concordant or discordant F508del homozygous sib pairs. Hum Genet (2002) 1.05
Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers. J Cyst Fibros (2004) 1.05
The TNFalpha receptor TNFRSF1A and genes encoding the amiloride-sensitive sodium channel ENaC as modulators in cystic fibrosis. Hum Genet (2006) 1.05
Interclonal gradient of virulence in the Pseudomonas aeruginosa pangenome from disease and environment. Environ Microbiol (2014) 1.04
Clonal epidemiology of Pseudomonas aeruginosa in cystic fibrosis. Int J Med Microbiol (2010) 1.04
Domains in biological membranes. Exp Cell Res (2009) 1.03
Alpha-kinase 1, a new component in apical protein transport. J Biol Chem (2005) 1.02
Genometa--a fast and accurate classifier for short metagenomic shotgun reads. PLoS One (2012) 1.02
Basic protocol for transepithelial nasal potential difference measurements. J Cyst Fibros (2004) 1.01
Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption. Hum Mutat (2006) 1.01
Pseudomonas aeruginosa population biology in chronic obstructive pulmonary disease. J Infect Dis (2009) 1.01
Distinct cytoskeletal tracks direct individual vesicle populations to the apical membrane of epithelial cells. Curr Biol (2003) 0.99
Pulmonary surfactant, lung function, and endobronchial inflammation in cystic fibrosis. Am J Respir Crit Care Med (2004) 0.99
Fitness of isogenic colony morphology variants of Pseudomonas aeruginosa in murine airway infection. PLoS One (2008) 0.99
Annexin II is required for apical transport in polarized epithelial cells. J Biol Chem (2003) 0.99
Genome Sequence of Burkholderia cenocepacia H111, a Cystic Fibrosis Airway Isolate. Genome Announc (2014) 0.98
GeneChip expression analysis of the VqsR regulon of Pseudomonas aeruginosa TB. FEMS Microbiol Lett (2005) 0.98
Multiple roles of Pseudomonas aeruginosa TBCF10839 PilY1 in motility, transport and infection. Mol Microbiol (2008) 0.97
Molecular epidemiology of chronic Pseudomonas aeruginosa airway infections in cystic fibrosis. PLoS One (2012) 0.97
Intraclonal diversity of the Pseudomonas aeruginosa cystic fibrosis airway isolates TBCF10839 and TBCF121838: distinct signatures of transcriptome, proteome, metabolome, adherence and pathogenicity despite an almost identical genome sequence. Environ Microbiol (2012) 0.97
Antibiotic treatment of CF lung disease: from bench to bedside. J Cyst Fibros (2011) 0.96
Crystal structure of the electron transfer complex rubredoxin rubredoxin reductase of Pseudomonas aeruginosa. Proc Natl Acad Sci U S A (2007) 0.95
The risk, prevention, and outcome of cytomegalovirus after pediatric lung transplantation. Transplantation (2009) 0.94
Accumulation of ceramide in the trachea and intestine of cystic fibrosis mice causes inflammation and cell death. Biochem Biophys Res Commun (2010) 0.94
Transcoronary delivery of bone marrow cells to the infarcted murine myocardium: feasibility, cellular kinetics, and improvement in cardiac function. Basic Res Cardiol (2006) 0.94
A novel sorting signal for intracellular localization is present in the S protein of a porcine coronavirus but absent from severe acute respiratory syndrome-associated coronavirus. J Biol Chem (2004) 0.93
EuroCareCF quality assessment of diagnostic microbiology of cystic fibrosis isolates. J Clin Microbiol (2009) 0.93
The SeqWord Genome Browser: an online tool for the identification and visualization of atypical regions of bacterial genomes through oligonucleotide usage. BMC Bioinformatics (2008) 0.93
Neuropeptide Y (NPY) cleaving enzymes: structural and functional homologues of dipeptidyl peptidase 4. Peptides (2007) 0.93
Binding of Par-4 to the actin cytoskeleton is essential for Par-4/Dlk-mediated apoptosis. Exp Cell Res (2005) 0.93
Functional analysis of F508del CFTR in native human colon. Biochim Biophys Acta (2010) 0.92
A new role of the complement system: C3 provides protection in a mouse model of lung infection with intracellular Chlamydia psittaci. PLoS One (2012) 0.92
Developing an international Pseudomonas aeruginosa reference panel. Microbiologyopen (2013) 0.91
Murine pulmonary infection with Listeria monocytogenes: differential susceptibility of BALB/c, C57BL/6 and DBA/2 mice. Microbes Infect (2005) 0.91
Complete Genome Sequence of Persistent Cystic Fibrosis Isolate Pseudomonas aeruginosa Strain RP73. Genome Announc (2013) 0.91
Ex vivo transcriptional profiling reveals a common set of genes important for the adaptation of Pseudomonas aeruginosa to chronically infected host sites. Environ Microbiol (2012) 0.91
In silico comparison of pKLC102-like genomic islands of Pseudomonas aeruginosa. FEMS Microbiol Lett (2007) 0.91
Impaired trafficking and subcellular localization of a mutant lactase associated with congenital lactase deficiency. Gastroenterology (2009) 0.90
Unexpected high digestion rate of cooked starch by the Ct-maltase-glucoamylase small intestine mucosal α-glucosidase subunit. PLoS One (2012) 0.90
In-vivo expression profiling of Pseudomonas aeruginosa infections reveals niche-specific and strain-independent transcriptional programs. PLoS One (2011) 0.89