Published in Am J Respir Crit Care Med on May 11, 2006
Structure, function, and post-translational regulation of the catalytic and modifier subunits of glutamate cysteine ligase. Mol Aspects Med (2008) 1.92
Environmental toxicity, redox signaling and lung inflammation: the role of glutathione. Mol Aspects Med (2008) 1.56
Recent advances in genetic predisposition to clinical acute lung injury. Am J Physiol Lung Cell Mol Physiol (2009) 1.34
Update on gene modifiers in cystic fibrosis. Curr Opin Pulm Med (2008) 1.29
Interaction between a novel TGFB1 haplotype and CFTR genotype is associated with improved lung function in cystic fibrosis. Hum Mol Genet (2008) 1.12
Strategies for identifying modifier genes in cystic fibrosis. Proc Am Thorac Soc (2007) 0.99
Genetic variation in the glutathione synthesis pathway, air pollution, and children's lung function growth. Am J Respir Crit Care Med (2010) 0.97
Update in cystic fibrosis 2006. Am J Respir Crit Care Med (2007) 0.93
Lung glutathione adaptive responses to cigarette smoke exposure. Respir Res (2011) 0.92
Genetic interaction of GSH metabolic pathway genes in cystic fibrosis. BMC Med Genet (2013) 0.90
Functional significance of the GAG trinucleotide-repeat polymorphism in the gene for the catalytic subunit of gamma-glutamylcysteine ligase. Free Radic Biol Med (2008) 0.89
Genetic influences on cystic fibrosis lung disease severity. Front Pharmacol (2013) 0.88
Disease modifying genes in cystic fibrosis: therapeutic option or one-way road? Naunyn Schmiedebergs Arch Pharmacol (2006) 0.87
Reciprocal regulation of TGF-β and reactive oxygen species: A perverse cycle for fibrosis. Redox Biol (2015) 0.83
A functional trinucleotide repeat polymorphism in the 5'-untranslated region of the glutathione biosynthetic gene GCLC is associated with increased risk for lung and aerodigestive tract cancers. Mol Carcinog (2012) 0.79
Polymorphisms in the glutathione pathway modulate cystic fibrosis severity: a cross-sectional study. BMC Med Genet (2014) 0.79
Lack of Association Between Haptoglobin Phenotype and Cystic Fibrosis Outcomes. Lung (2015) 0.75
Association of GCLM -588C/T and GCLC -129T/C Promoter Polymorphisms of Genes Coding the Subunits of Glutamate Cysteine Ligase with Ischemic Heart Disease Development in Kazakhstan Population. Dis Markers (2017) 0.75
Population stratification and spurious allelic association. Lancet (2003) 10.53
Early pulmonary inflammation in infants with cystic fibrosis. Am J Respir Crit Care Med (1995) 6.56
Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell (1993) 6.25
Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med (2005) 5.51
The diagnosis of cystic fibrosis: a consensus statement. Cystic Fibrosis Foundation Consensus Panel. J Pediatr (1998) 5.08
The relation between genotype and phenotype in cystic fibrosis--analysis of the most common mutation (delta F508). N Engl J Med (1990) 4.70
Independent genetic determinants of pancreatic and pulmonary status in cystic fibrosis. Lancet (1990) 3.27
Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation. Am J Respir Crit Care Med (1994) 3.24
The spectrum of cystic fibrosis mutations. Trends Genet (1992) 2.88
Oxidative stress and regulation of glutathione in lung inflammation. Eur Respir J (2000) 2.63
Genetic determination of exocrine pancreatic function in cystic fibrosis. Am J Hum Genet (1992) 2.48
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet (2003) 2.36
Cystic fibrosis transmembrane conductance regulator function is suppressed in cigarette smokers. Am J Respir Crit Care Med (2006) 2.34
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations. N Engl J Med (1994) 2.25
Association of mannose-binding lectin gene heterogeneity with severity of lung disease and survival in cystic fibrosis. J Clin Invest (1999) 2.24
TGF-beta(1) genotype and accelerated decline in lung function of patients with cystic fibrosis. Thorax (2000) 2.14
Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis. BMJ (1995) 2.12
Systemic deficiency of glutathione in cystic fibrosis. J Appl Physiol (1985) (1993) 1.88
Endothelial nitric oxide synthase variants in cystic fibrosis lung disease. Am J Respir Crit Care Med (2002) 1.87
The regulation of hepatic glutathione. Annu Rev Pharmacol Toxicol (1985) 1.84
Liver disease in pediatric patients with cystic fibrosis is associated with glutathione S-transferase P1 polymorphism. Hepatology (2002) 1.62
Contribution of genetic factors other than CFTR to disease severity in cystic fibrosis. Thorax (1998) 1.60
3849+10 kb C-->T mutation and disease severity in cystic fibrosis. Lancet (1995) 1.57
End-organ dysfunction in cystic fibrosis: association with angiotensin I converting enzyme and cytokine gene polymorphisms. Am J Respir Crit Care Med (2003) 1.52
Glutathione permeability of CFTR. Am J Physiol (1998) 1.48
CFTR directly mediates nucleotide-regulated glutathione flux. EMBO J (2003) 1.45
Abnormal glutathione transport in cystic fibrosis airway epithelia. Am J Physiol (1999) 1.36
Categories of deltaF508 homozygous cystic fibrosis twin and sibling pairs with distinct phenotypic characteristics. Twin Res (2000) 1.29
Five percent of normal cystic fibrosis transmembrane conductance regulator mRNA ameliorates the severity of pulmonary disease in cystic fibrosis. Am J Respir Cell Mol Biol (2002) 1.26
Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress. Eur Respir J (1996) 1.24
Oxidant stress suppresses CFTR expression. Am J Physiol Cell Physiol (2005) 1.19
Pulmonary oxidative stress response in young children with cystic fibrosis. Thorax (1997) 1.18
Neutrophil-derived long-lived oxidants in cystic fibrosis sputum. Am J Respir Crit Care Med (1995) 1.17
Improvement of alveolar glutathione and lung function but not oxidative state in cystic fibrosis. Am J Respir Crit Care Med (2004) 1.05
Evidence for functionally significant polymorphism of human glutamate cysteine ligase catalytic subunit: association with glutathione levels and drug resistance in the National Cancer Institute tumor cell line panel. Toxicol Sci (2001) 1.02
A cystic fibrosis mutation associated with mild lung disease. N Engl J Med (1995) 1.00
Cystic fibrosis, disease severity, and a macrophage migration inhibitory factor polymorphism. Am J Respir Crit Care Med (2005) 0.98
Identification of a splice site mutation (2789 +5 G > A) associated with small amounts of normal CFTR mRNA and mild cystic fibrosis. Hum Mutat (1997) 0.97
Synthetic chloride channel restores glutathione secretion in cystic fibrosis airway epithelia. Am J Physiol Lung Cell Mol Physiol (2001) 0.95
Seeking modifier genes in cystic fibrosis. Am J Respir Crit Care Med (2003) 0.90
Glutathione-S-transferase M1, M3, P1 and T1 polymorphisms and severity of lung disease in children with cystic fibrosis. Pharmacogenetics (2004) 0.90
An alpha1-antitrypsin enhancer polymorphism is a genetic modifier of pulmonary outcome in cystic fibrosis. Eur J Hum Genet (2001) 0.90
Aberrant free radical activity in cystic fibrosis. Clin Chim Acta (1989) 0.88
Assignment of the gene (GLCLC) that encodes the heavy subunit of gamma-glutamylcysteine synthetase to human chromosome 6. Cytogenet Cell Genet (1995) 0.87
Variable severity of pulmonary disease in adults with identical cystic fibrosis mutations. Chest (1992) 0.85
Genetic mapping of GLCLC, the human gene encoding the catalytic subunit of gamma-glutamyl-cysteine synthetase, to chromosome band 6p12 and characterization of a polymorphic trinucleotide repeat within its 5' untranslated region. Cytogenet Cell Genet (1996) 0.83
Modifier genes and variation in cystic fibrosis. Respir Res (2001) 0.82
RIP3, an energy metabolism regulator that switches TNF-induced cell death from apoptosis to necrosis. Science (2009) 9.29
Standardizing global gene expression analysis between laboratories and across platforms. Nat Methods (2005) 6.23
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med (2005) 5.51
Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome. Nat Genet (2007) 3.22
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
Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J Respir Crit Care Med (2002) 2.50
Selection of reference genes for gene expression studies in human neutrophils by real-time PCR. BMC Mol Biol (2005) 2.44
Effect of genotype on phenotype and mortality in cystic fibrosis: a retrospective cohort study. Lancet (2003) 2.36
Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. Am J Respir Crit Care Med (2003) 2.26
Direct sampling of cystic fibrosis lungs indicates that DNA-based analyses of upper-airway specimens can misrepresent lung microbiota. Proc Natl Acad Sci U S A (2012) 2.22
Fentanyl-induced chest wall rigidity. Chest (2013) 2.05
Survival after lung volume reduction in chronic obstructive pulmonary disease: insights from small airway pathology. Am J Respir Crit Care Med (2007) 2.04
Association between pulmonary function and sputum biomarkers in cystic fibrosis. Am J Respir Crit Care Med (2007) 2.03
The role of matrix metalloproteinase polymorphisms in the rate of decline in lung function. Hum Mol Genet (2002) 1.99
Effectiveness of screening examinations to detect unrecognized exercise-induced bronchoconstriction. J Pediatr (2002) 1.96
Structure, function, and post-translational regulation of the catalytic and modifier subunits of glutamate cysteine ligase. Mol Aspects Med (2008) 1.92
Co-regulation of CYP3A4 and CYP3A5 and contribution to hepatic and intestinal midazolam metabolism. Mol Pharmacol (2002) 1.91
Studying communication about end-of-life care during the ICU family conference: development of a framework. J Crit Care (2002) 1.90
Prevalence of neutralizing antibodies against adeno-associated virus (AAV) types 2, 5, and 6 in cystic fibrosis and normal populations: Implications for gene therapy using AAV vectors. Hum Gene Ther (2006) 1.78
Antioxidant gene polymorphisms and susceptibility to a rapid decline in lung function in smokers. Am J Respir Crit Care Med (2002) 1.76
Polymorphisms in the estrogen synthesis and metabolism pathways and symptoms during the menopausal transition: observations from the Seattle Midlife Women's Health Study. Menopause (2006) 1.74
Association of Vitamin K epoxide reductase complex 1 (VKORC1) variants with warfarin dose in a Hong Kong Chinese patient population. Pharmacogenet Genomics (2005) 1.72
Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis. Pediatr Pulmonol (2002) 1.67
Disparities in access to lung transplantation for patients with cystic fibrosis by socioeconomic status. Am J Respir Crit Care Med (2012) 1.64
Childhood brain tumors, residential insecticide exposure, and pesticide metabolism genes. Environ Health Perspect (2010) 1.62
Genetic polymorphisms in the catechol estrogen metabolism pathway and breast cancer risk. Cancer Epidemiol Biomarkers Prev (2009) 1.62
Risk of post-lung transplant renal dysfunction in adults with cystic fibrosis. Chest (2012) 1.60
Glutamate cysteine ligase modifier subunit deficiency and gender as determinants of acetaminophen-induced hepatotoxicity in mice. Toxicol Sci (2007) 1.60
Respiratory outbreak of Mycobacterium abscessus subspecies massiliense in a lung transplant and cystic fibrosis center. Am J Respir Crit Care Med (2012) 1.59
Association of endothelial lipase gene (LIPG) haplotypes with high-density lipoprotein cholesterol subfractions and apolipoprotein AI plasma levels in Japanese Americans. Atherosclerosis (2005) 1.58
Spectral index for assessment of differential protein expression in shotgun proteomics. J Proteome Res (2008) 1.55
Effects of bronchoconstriction, minute ventilation, and deep inspiration on the composition of exhaled breath condensate. Chest (2010) 1.54
Risk factors for chronic kidney disease in adults with cystic fibrosis. Am J Respir Crit Care Med (2011) 1.53
Interlaboratory evaluation of rodent pulmonary responses to engineered nanomaterials: the NIEHS Nano GO Consortium. Environ Health Perspect (2013) 1.52
Modification of neurobehavioral effects of mercury by genetic polymorphisms of metallothionein in children. Neurotoxicol Teratol (2013) 1.50
Molecular signature of smoking in human lung tissues. Cancer Res (2012) 1.50
Detecting Stenotrophomonas maltophilia does not reduce survival of patients with cystic fibrosis. Am J Respir Crit Care Med (2002) 1.46
Fluorescence-based microtiter plate assay for glutamate-cysteine ligase activity. Anal Biochem (2003) 1.46
Association of CYP17, CYP19, CYP1B1, and COMT polymorphisms with serum and urinary sex hormone concentrations in postmenopausal women. Cancer Epidemiol Biomarkers Prev (2004) 1.35
Airway wall remodeling: friend or foe? J Appl Physiol (1985) (2003) 1.33
CFTR genotype as a predictor of prognosis in cystic fibrosis. Chest (2006) 1.31
Variable regulation of glutamate cysteine ligase subunit proteins affects glutathione biosynthesis in response to oxidative stress. Arch Biochem Biophys (2004) 1.29
Inflammatory basis of exercise-induced bronchoconstriction. Am J Respir Crit Care Med (2005) 1.28
Recent advances in asthma genetics. Respir Res (2008) 1.23
High-level relatedness among Mycobacterium abscessus subsp. massiliense strains from widely separated outbreaks. Emerg Infect Dis (2014) 1.21
Induction of glutathione synthesis in macrophages by oxidized low-density lipoproteins is mediated by consensus antioxidant response elements. Circ Res (2003) 1.20
Glutathione S-transferase variants and their interaction with smoking on lung function. Am J Respir Crit Care Med (2004) 1.19
TGFbeta1-induced suppression of glutathione antioxidant defenses in hepatocytes: caspase-dependent post-translational and caspase-independent transcriptional regulatory mechanisms. FASEB J (2003) 1.18
The association between a genetic polymorphism of coproporphyrinogen oxidase, dental mercury exposure and neurobehavioral response in humans. Neurotoxicol Teratol (2005) 1.17
Diesel exhaust inhalation and assessment of peripheral blood mononuclear cell gene transcription effects: an exploratory study of healthy human volunteers. Inhal Toxicol (2007) 1.17
Chronic low-level mercury exposure, BDNF polymorphism, and associations with cognitive and motor function. Neurotoxicol Teratol (2005) 1.15
Serum cholinesterase inhibition in relation to paraoxonase-1 (PON1) status among organophosphate-exposed agricultural pesticide handlers. Environ Health Perspect (2009) 1.14
Airway immunopathology of asthma with exercise-induced bronchoconstriction. J Allergy Clin Immunol (2005) 1.12
The relationship between telomere length and mortality in chronic obstructive pulmonary disease (COPD). PLoS One (2012) 1.12
Changes in atherosclerotic plaques induced by inhalation of diesel exhaust. Atherosclerosis (2011) 1.12
Neurotoxicity of a polybrominated diphenyl ether mixture (DE-71) in mouse neurons and astrocytes is modulated by intracellular glutathione levels. Toxicol Appl Pharmacol (2008) 1.10
Glutathione-s-transferase M1 and T1 polymorphisms and associations with type 1 diabetes age-at-onset. Autoimmunity (2005) 1.09
Genetic risk factors of chronic obstructive pulmonary disease. Swiss Med Wkly (2002) 1.08
Recombinant erythropoietin is neuroprotective in a novel mouse oxidative injury model. Dev Neurosci (2007) 1.08
No detectable improvements in cystic fibrosis transmembrane conductance regulator by nasal aminoglycosides in patients with cystic fibrosis with stop mutations. Am J Respir Cell Mol Biol (2007) 1.08
Organophosphorus insecticides chlorpyrifos and diazinon and oxidative stress in neuronal cells in a genetic model of glutathione deficiency. Toxicol Appl Pharmacol (2006) 1.07
Microarray analysis of high-dose recombinant erythropoietin treatment of unilateral brain injury in neonatal mouse hippocampus. Pediatr Res (2009) 1.06
Glutamate-cysteine ligase attenuates TNF-induced mitochondrial injury and apoptosis. Free Radic Biol Med (2004) 1.05
Glutathione S-transferase M1, T1, and P1 polymorphisms and Parkinson's disease. Neurosci Lett (2003) 1.05
Association of apolipoprotein A5 variants with LDL particle size and triglyceride in Japanese Americans. Biochim Biophys Acta (2004) 1.05
Multicenter study of acetaminophen hepatotoxicity reveals the importance of biological endpoints in genomic analyses. Toxicol Sci (2007) 1.04
Caspase-3-Dependent Cleavage of the Glutamate-L-Cysteine Ligase Catalytic Subunit during Apoptotic Cell Death. Am J Pathol (2002) 1.04
Rapid activation of glutamate cysteine ligase following oxidative stress. J Biol Chem (2010) 1.04
Transglutaminase 2, a novel regulator of eicosanoid production in asthma revealed by genome-wide expression profiling of distinct asthma phenotypes. PLoS One (2010) 1.04
Genome-wide association studies for discovery of genes involved in asthma. Respirology (2011) 1.03
The association between genetic polymorphisms of coproporphyrinogen oxidase and an atypical porphyrinogenic response to mercury exposure in humans. Toxicol Appl Pharmacol (2005) 1.02
Acetaminophen-induced liver injury is attenuated in male glutamate-cysteine ligase transgenic mice. J Biol Chem (2006) 1.02
Mitochondrial-targeted peptide rapidly improves mitochondrial energetics and skeletal muscle performance in aged mice. Aging Cell (2013) 1.02
Repeated developmental exposure of mice to chlorpyrifos oxon is associated with paraoxonase 1 (PON1)-modulated effects on cerebellar gene expression. Toxicol Sci (2011) 1.02
Mapping the lung proteome in cystic fibrosis. J Proteome Res (2009) 1.01
Erythropoietin induces heme oxygenase-1 expression and attenuates oxidative stress. Biochem Biophys Res Commun (2007) 1.01
The cycad genotoxin MAM modulates brain cellular pathways involved in neurodegenerative disease and cancer in a DNA damage-linked manner. PLoS One (2011) 1.01
Two major facilitator superfamily sugar transporters from Trichoderma reesei and their roles in induction of cellulase biosynthesis. J Biol Chem (2013) 1.00
Matrix metalloproteinase expression by human alveolar macrophages in relation to emphysema. COPD (2008) 1.00
Polymorphisms in the IL13, IL13RA1, and IL4RA genes and rate of decline in lung function in smokers. Am J Respir Cell Mol Biol (2003) 0.99
Functional genetic variation in NFKBIA and susceptibility to childhood asthma, bronchiolitis, and bronchopulmonary dysplasia. J Immunol (2013) 0.98
GST, NAT1, CYP1A1 polymorphisms and risk of esophageal and gastric adenocarcinomas. Cancer Detect Prev (2007) 0.98
Glutathione levels modulate domoic acid induced apoptosis in mouse cerebellar granule cells. Toxicol Sci (2007) 0.98
Chronic low-level mercury exposure, BDNF polymorphism, and associations with self-reported symptoms and mood. Toxicol Sci (2004) 0.98
An official multi-society statement: the role of clinical research results in the practice of critical care medicine. Am J Respir Crit Care Med (2012) 0.97
Modulating GSH synthesis using glutamate cysteine ligase transgenic and gene-targeted mice. Drug Metab Rev (2008) 0.97
Causal and synthetic associations of variants in the SERPINA gene cluster with alpha1-antitrypsin serum levels. PLoS Genet (2013) 0.97
TLR5 as an anti-inflammatory target and modifier gene in cystic fibrosis. J Immunol (2010) 0.97
Attenuated progression of diet-induced steatohepatitis in glutathione-deficient mice. Lab Invest (2010) 0.97
Influenza-associated cystic fibrosis pulmonary exacerbations. Chest (2009) 0.97
Risk of brain tumors in children and susceptibility to organophosphorus insecticides: the potential role of paraoxonase (PON1). Environ Health Perspect (2005) 0.96
Genetic polymorphisms in dopamine-related genes and smoking cessation in women: a prospective cohort study. Behav Brain Funct (2007) 0.96
Fellows as teachers: the teacher-assistant experience during pulmonary subspecialty training. Chest (2005) 0.95
Telomere length and chronic obstructive pulmonary disease: evidence of accelerated aging. J Am Geriatr Soc (2009) 0.95
Quality of life in adolescents with mild asthma. Pediatr Pulmonol (2003) 0.95