Published in Am J Respir Crit Care Med on October 09, 2003
Cystic fibrosis genetics: from molecular understanding to clinical application. Nat Rev Genet (2014) 1.70
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med (2006) 1.44
Clinical practice and genetic counseling for cystic fibrosis and CFTR-related disorders. Genet Med (2008) 1.29
Effects of gender and age at diagnosis on disease progression in long-term survivors of cystic fibrosis. Am J Respir Crit Care Med (2010) 1.18
Cystic fibrosis patients, infertile men, and their noses. Am J Respir Crit Care Med (2004) 0.75
Normalization of obesity-associated insulin resistance through immunotherapy. Nat Med (2009) 8.15
Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med (2011) 6.74
Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med (2005) 5.51
Genome-wide association and linkage identify modifier loci of lung disease severity in cystic fibrosis at 11p13 and 20q13.2. Nat Genet (2011) 3.02
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet (2013) 2.91
Rac1 deletion in mouse neutrophils has selective effects on neutrophil functions. J Immunol (2003) 2.59
Reactive oxygen and nitrogen species as signaling molecules regulating neutrophil function. Free Radic Biol Med (2006) 2.48
Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med (2006) 2.34
Rac1 is the small GTPase responsible for regulating the neutrophil chemotaxis compass. Blood (2004) 2.06
Shwachman-Diamond syndrome: report from an international conference. J Pediatr (2002) 1.99
Cystic fibrosis mouse models. Am J Respir Cell Mol Biol (2006) 1.98
Transepithelial migration of neutrophils: mechanisms and implications for acute lung injury. Am J Respir Cell Mol Biol (2008) 1.93
β-adrenergic sweat secretion as a diagnostic test for cystic fibrosis. Am J Respir Crit Care Med (2012) 1.92
Genetic modifiers of liver disease in cystic fibrosis. JAMA (2009) 1.88
High-dose ibuprofen in cystic fibrosis: Canadian safety and effectiveness trial. J Pediatr (2007) 1.86
Proteinase-activated receptor-1 mediates elastase-induced apoptosis of human lung epithelial cells. Am J Respir Cell Mol Biol (2005) 1.83
Genotype and phenotype correlations in patients with cystic fibrosis and pancreatitis. Gastroenterology (2002) 1.80
PhenCode: connecting ENCODE data with mutations and phenotype. Hum Mutat (2007) 1.75
Oxidative stress and acute lung injury. Am J Respir Cell Mol Biol (2003) 1.75
Serum VEGF-D a concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial. Lancet Respir Med (2013) 1.69
Matrix metalloproteinase induction of Rac1b, a key effector of lung cancer progression. Sci Transl Med (2012) 1.67
The remote ischemic preconditioning stimulus modifies inflammatory gene expression in humans. Physiol Genomics (2004) 1.63
Complex two-gene modulation of lung disease severity in children with cystic fibrosis. J Clin Invest (2008) 1.57
Glucose intolerance in children with cystic fibrosis. J Pediatr (2003) 1.51
Primary sclerosing cholangitis in childhood is associated with abnormalities in cystic fibrosis-mediated chloride channel function. J Pediatr (2007) 1.48
Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphans. Am J Respir Cell Mol Biol (2006) 1.47
A systematic analysis of intronic sequences downstream of 5' splice sites reveals a widespread role for U-rich motifs and TIA1/TIAL1 proteins in alternative splicing regulation. Genome Res (2008) 1.44
Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials. Am J Respir Crit Care Med (2006) 1.44
Neutrophil granule contents in the pathogenesis of lung injury. Curr Opin Hematol (2006) 1.43
Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. Respir Med (2009) 1.36
Type of CFTR mutation determines risk of pancreatitis in patients with cystic fibrosis. Gastroenterology (2010) 1.33
Draft consensus guidelines for diagnosis and treatment of Shwachman-Diamond syndrome. Ann N Y Acad Sci (2011) 1.33
Lung inflammation as a therapeutic target in cystic fibrosis. Am J Respir Cell Mol Biol (2004) 1.28
Human neutrophil peptides induce interleukin-8 production through the P2Y6 signaling pathway. Blood (2005) 1.27
Proteases and lung injury. Crit Care Med (2003) 1.27
Air travel in women with lymphangioleiomyomatosis. Thorax (2006) 1.27
Matrix metalloproteinase 3 is a mediator of pulmonary fibrosis. Am J Pathol (2011) 1.27
Multiple apical plasma membrane constituents are associated with susceptibility to meconium ileus in individuals with cystic fibrosis. Nat Genet (2012) 1.26
Neutrophil transmigration triggers repair of the lung epithelium via beta-catenin signaling. Proc Natl Acad Sci U S A (2011) 1.25
Neutrophil apoptosis: a marker of disease severity in sepsis and sepsis-induced acute respiratory distress syndrome. Crit Care (2006) 1.23
Control of vesicle fusion by a tyrosine phosphatase. Nat Cell Biol (2004) 1.21
Clinical manifestations of cystic fibrosis among patients with diagnosis in adulthood. Chest (2004) 1.18
Characterization of disease-associated mutations affecting an exonic splicing enhancer and two cryptic splice sites in exon 13 of the cystic fibrosis transmembrane conductance regulator gene. Hum Mol Genet (2003) 1.18
L-selectin stimulation enhances functional expression of surface CXCR4 in lymphocytes: implications for cellular activation during adhesion and migration. Blood (2003) 1.17
Staphylococcus aureus sigma B-dependent emergence of small-colony variants and biofilm production following exposure to Pseudomonas aeruginosa 4-hydroxy-2-heptylquinoline-N-oxide. BMC Microbiol (2010) 1.16
Dysfunctional cystic fibrosis transmembrane conductance regulator inhibits phagocytosis of apoptotic cells with proinflammatory consequences. Am J Physiol Lung Cell Mol Physiol (2009) 1.14
Two-tiered immunoreactive trypsinogen-based newborn screening for cystic fibrosis in Colorado: screening efficacy and diagnostic outcomes. J Pediatr (2005) 1.14
Murine hepatitis virus strain 1 produces a clinically relevant model of severe acute respiratory syndrome in A/J mice. J Virol (2006) 1.13
Tumor necrosis factor-α accelerates the resolution of established pulmonary fibrosis in mice by targeting profibrotic lung macrophages. Am J Respir Cell Mol Biol (2014) 1.12
Role of innate immune cells and their products in lung immunopathology. Int J Biochem Cell Biol (2008) 1.10
Unraveling the complex genetic model for cystic fibrosis: pleiotropic effects of modifier genes on early cystic fibrosis-related morbidities. Hum Genet (2013) 1.10
Remote ischemic preconditioning decreases adhesion and selectively modifies functional responses of human neutrophils. J Surg Res (2010) 1.08
Modifier gene study of meconium ileus in cystic fibrosis: statistical considerations and gene mapping results. Hum Genet (2009) 1.08
The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis. Hum Genet (2005) 1.08
Clonal evolution in marrows of patients with Shwachman-Diamond syndrome: a prospective 5-year follow-up study. Exp Hematol (2002) 1.06
Age and sex dimorphisms contribute to the severity of bleomycin-induced lung injury and fibrosis. Am J Physiol Lung Cell Mol Physiol (2011) 1.06
Translocation of the tetraspanin CD63 in association with human eosinophil mediator release. Blood (2002) 1.04
Leukocyte elastase induces epithelial apoptosis: role of mitochondial permeability changes and Akt. Am J Physiol Gastrointest Liver Physiol (2004) 1.03
CD44 is a phagocytic receptor. Blood (2006) 1.03
Leukocyte elastase induces lung epithelial apoptosis via a PAR-1-, NF-kappaB-, and p53-dependent pathway. Am J Respir Cell Mol Biol (2009) 1.02
Analysis of the cystic fibrosis lung microbiota via serial Illumina sequencing of bacterial 16S rRNA hypervariable regions. PLoS One (2012) 1.00
A novel lung disease phenotype adjusted for mortality attrition for cystic fibrosis genetic modifier studies. Pediatr Pulmonol (2011) 1.00
Zinc chelators inhibit eotaxin, RANTES, and MCP-1 production in stimulated human airway epithelium and fibroblasts. Am J Physiol Lung Cell Mol Physiol (2003) 1.00
Signalling platforms that modulate the inflammatory response: new targets for drug development. Nat Rev Drug Discov (2006) 1.00
Role of β-catenin-regulated CCN matricellular proteins in epithelial repair after inflammatory lung injury. Am J Physiol Lung Cell Mol Physiol (2013) 0.99
Rare lung disease II: pulmonary alveolar proteinosis. Can Respir J (2008) 0.99
Role of PAR2 in murine pulmonary pseudomonal infection. Am J Physiol Lung Cell Mol Physiol (2007) 0.98
CD44-mediated phagocytosis induces inside-out activation of complement receptor-3 in murine macrophages. Blood (2007) 0.97
TLR5 as an anti-inflammatory target and modifier gene in cystic fibrosis. J Immunol (2010) 0.97
How useful is fecal pancreatic elastase 1 as a marker of exocrine pancreatic disease? J Pediatr (2002) 0.97
The fibroproliferative response in acute respiratory distress syndrome: mechanisms and clinical significance. Eur Respir J (2013) 0.95
Increased prevalence of CFTR mutations and variants and decreased chloride secretion in primary sclerosing cholangitis. Hum Genet (2003) 0.95
On, around, and through: neutrophil-endothelial interactions in innate immunity. Physiology (Bethesda) (2011) 0.95
Inactivation of macrophage Rab7 by Burkholderia cenocepacia. J Innate Immun (2010) 0.94
Tyrosine phosphatase MEG2 modulates murine development and platelet and lymphocyte activation through secretory vesicle function. J Exp Med (2005) 0.94
SHP-2 modulates interleukin-1-induced Ca2+ flux and ERK activation via phosphorylation of phospholipase Cgamma1. J Biol Chem (2004) 0.94
Matrix metalloproteinases and protein tyrosine kinases: potential novel targets in acute lung injury and ARDS. Chest (2014) 0.93
Protein-tyrosine phosphatase MEG2 is expressed by human neutrophils. Localization to the phagosome and activation by polyphosphoinositides. J Biol Chem (2001) 0.92
Feasibility of internet-based health-related quality of life data collection in a large patient cohort. J Med Internet Res (2010) 0.92
Pneumococcal vaccination programs and the burden of invasive pneumococcal disease in Ontario, Canada, 1995-2011. Vaccine (2013) 0.91
Docosahexaenoic acid derivative prevents inflammation and hyperreactivity in lung: implication of PKC-Potentiated inhibitory protein for heterotrimeric myosin light chain phosphatase of 17 kD in asthma. Am J Respir Cell Mol Biol (2010) 0.91
The prostanoid 15-deoxy-Δ12,14-prostaglandin-j2 reduces lung inflammation and protects mice against lethal influenza infection. J Infect Dis (2012) 0.91
Outcome in cystic fibrosis liver disease. Am J Gastroenterol (2010) 0.90
SigB is a dominant regulator of virulence in Staphylococcus aureus small-colony variants. PLoS One (2013) 0.90
Lysosomotropic drugs inhibit maturation of transforming growth factor-beta. Can J Physiol Pharmacol (2008) 0.90
IL-1β enhances cell adhesion to degraded fibronectin. FASEB J (2012) 0.89