Published in Mol Ther on September 01, 2004
Mechanisms of the noxious inflammatory cycle in cystic fibrosis. Respir Res (2009) 1.16
Influence of cystic fibrosis transmembrane conductance regulator on gene expression in response to Pseudomonas aeruginosa infection of human bronchial epithelial cells. Infect Immun (2005) 1.03
Defective acid sphingomyelinase pathway with Pseudomonas aeruginosa infection in cystic fibrosis. Am J Respir Cell Mol Biol (2009) 0.97
Oxidative stress causes IL8 promoter hyperacetylation in cystic fibrosis airway cell models. Am J Respir Cell Mol Biol (2008) 0.97
Changes in transcriptome of native nasal epithelium expressing F508del-CFTR and intersecting data from comparable studies. Respir Res (2013) 0.85
Murine model for cystic fibrosis bone disease demonstrates osteopenia and sex-related differences in bone formation. Pediatr Res (2009) 0.83
Does the F508-CFTR mutation induce a proinflammatory response in human airway epithelial cells? Am J Physiol Lung Cell Mol Physiol (2012) 0.82
A novel approach to analyze gene expression data demonstrates that the DeltaF508 mutation in CFTR downregulates the antigen presentation pathway. Am J Physiol Lung Cell Mol Physiol (2009) 0.82
Effect of polarized release of CXC-chemokines from wild-type and cystic fibrosis murine airway epithelial cells. Am J Respir Cell Mol Biol (2010) 0.81
Omics approaches in cystic fibrosis research: a focus on oxylipin profiling in airway secretions. Ann N Y Acad Sci (2012) 0.81
N-glycosylation augmentation of the cystic fibrosis epithelium improves Pseudomonas aeruginosa clearance. Am J Respir Cell Mol Biol (2010) 0.77
Cystic fibrosis transmembrane conductance regulator controls lung proteasomal degradation and nuclear factor-kappaB activity in conditions of oxidative stress. Am J Pathol (2008) 0.77
Normal and Cystic Fibrosis Human Bronchial Epithelial Cells Infected with Pseudomonas aeruginosa Exhibit Distinct Gene Activation Patterns. PLoS One (2015) 0.76
In silico search for modifier genes associated with pancreatic and liver disease in Cystic Fibrosis. PLoS One (2017) 0.75
Transcriptomic profile of cystic fibrosis patients identifies type I interferon response and ribosomal stalk proteins as potential modifiers of disease severity. PLoS One (2017) 0.75
Genomic responses in mouse models poorly mimic human inflammatory diseases. Proc Natl Acad Sci U S A (2013) 16.74
A network-based analysis of systemic inflammation in humans. Nature (2005) 12.60
Treatment of leber congenital amaurosis due to RPE65 mutations by ocular subretinal injection of adeno-associated virus gene vector: short-term results of a phase I trial. Hum Gene Ther (2008) 8.83
Human gene therapy for RPE65 isomerase deficiency activates the retinoid cycle of vision but with slow rod kinetics. Proc Natl Acad Sci U S A (2008) 7.16
The ClinSeq Project: piloting large-scale genome sequencing for research in genomic medicine. Genome Res (2009) 6.83
A genomic storm in critically injured humans. J Exp Med (2011) 6.67
Kaposi's sarcoma-associated herpesvirus encodes an ortholog of miR-155. J Virol (2007) 4.19
Analysis of the tyrosine kinome in melanoma reveals recurrent mutations in ERBB4. Nat Genet (2009) 3.80
Sustained transgene expression despite T lymphocyte responses in a clinical trial of rAAV1-AAT gene therapy. Proc Natl Acad Sci U S A (2009) 3.58
Application of genome-wide expression analysis to human health and disease. Proc Natl Acad Sci U S A (2005) 3.49
Whole blood and leukocyte RNA isolation for gene expression analyses. Physiol Genomics (2004) 3.22
Identification of cellular genes targeted by KSHV-encoded microRNAs. PLoS Pathog (2007) 3.16
TTC21B contributes both causal and modifying alleles across the ciliopathy spectrum. Nat Genet (2011) 3.06
Safety of recombinant adeno-associated virus type 2-RPE65 vector delivered by ocular subretinal injection. Mol Ther (2006) 2.13
Different roles of EIIABMan and EIIGlc in regulation of energy metabolism, biofilm development, and competence in Streptococcus mutans. J Bacteriol (2006) 2.00
Gray platelet syndrome: natural history of a large patient cohort and locus assignment to chromosome 3p. Blood (2010) 2.00
Compound heterozygosity for loss-of-function lysyl-tRNA synthetase mutations in a patient with peripheral neuropathy. Am J Hum Genet (2010) 1.96
Development of a genomic metric that can be rapidly used to predict clinical outcome in severely injured trauma patients. Crit Care Med (2013) 1.94
Phase I trial of intramuscular injection of a recombinant adeno-associated virus serotype 2 alphal-antitrypsin (AAT) vector in AAT-deficient adults. Hum Gene Ther (2006) 1.83
Cell-specific expression and pathway analyses reveal alterations in trauma-related human T cell and monocyte pathways. Proc Natl Acad Sci U S A (2006) 1.82
Safety in nonhuman primates of ocular AAV2-RPE65, a candidate treatment for blindness in Leber congenital amaurosis. Hum Gene Ther (2006) 1.81
alpha-1 antitrypsin inhibits caspase-3 activity, preventing lung endothelial cell apoptosis. Am J Pathol (2006) 1.81
Molecular characterization of the acute inflammatory response to infections with gram-negative versus gram-positive bacteria. Infect Immun (2003) 1.75
A novel antiapoptotic role for alpha1-antitrypsin in the prevention of pulmonary emphysema. Am J Respir Crit Care Med (2006) 1.72
Long-term, efficient inhibition of microRNA function in mice using rAAV vectors. Nat Methods (2012) 1.67
Controlling neuropathic pain by adeno-associated virus driven production of the anti-inflammatory cytokine, interleukin-10. Mol Pain (2005) 1.66
Repeated intrathecal injections of plasmid DNA encoding interleukin-10 produce prolonged reversal of neuropathic pain. Pain (2006) 1.66
A better understanding of why murine models of trauma do not recapitulate the human syndrome. Crit Care Med (2014) 1.62
ALDH isozymes downregulation affects cell growth, cell motility and gene expression in lung cancer cells. Mol Cancer (2008) 1.56
Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases. PLoS Genet (2011) 1.54
Characterization of a recombinant adeno-associated virus type 2 Reference Standard Material. Hum Gene Ther (2010) 1.51
Distinct transcriptional profiles characterize oral epithelium-microbiota interactions. Cell Microbiol (2005) 1.51
Incidental medical information in whole-exome sequencing. Pediatrics (2012) 1.48
DNA-dependent PK inhibits adeno-associated virus DNA integration. Proc Natl Acad Sci U S A (2004) 1.45
Gingival epithelial cell transcriptional responses to commensal and opportunistic oral microbial species. Infect Immun (2007) 1.44
Influence of BrpA on critical virulence attributes of Streptococcus mutans. J Bacteriol (2006) 1.42
Neonatal intraperitoneal or intravenous injections of recombinant adeno-associated virus type 8 transduce dorsal root ganglia and lower motor neurons. Hum Gene Ther (2008) 1.42
A phase II, double-blind, randomized, placebo-controlled clinical trial of tgAAVCF using maxillary sinus delivery in patients with cystic fibrosis with antrostomies. Hum Gene Ther (2002) 1.40
Commonality and differences in leukocyte gene expression patterns among three models of inflammation and injury. Physiol Genomics (2005) 1.40
NBEAL2 is mutated in gray platelet syndrome and is required for biogenesis of platelet α-granules. Nat Genet (2011) 1.38
Intramuscular administration of recombinant adeno-associated virus 2 alpha-1 antitrypsin (rAAV-SERPINA1) vectors in a nonhuman primate model: safety and immunologic aspects. Mol Ther (2002) 1.34
Analysis of factorial time-course microarrays with application to a clinical study of burn injury. Proc Natl Acad Sci U S A (2010) 1.33
Systemic overexpression of IL-10 induces CD4+CD25+ cell populations in vivo and ameliorates type 1 diabetes in nonobese diabetic mice in a dose-dependent fashion. J Immunol (2003) 1.32
The addition of tumor necrosis factor plus beta interferon induces a novel synergistic antiviral state against poxviruses in primary human fibroblasts. J Virol (2008) 1.32
MicroRNA-regulated, systemically delivered rAAV9: a step closer to CNS-restricted transgene expression. Mol Ther (2010) 1.30
Comparison of longitudinal leukocyte gene expression after burn injury or trauma-hemorrhage in mice. Physiol Genomics (2007) 1.30
Improved method of recombinant AAV2 delivery for systemic targeted gene therapy. Mol Ther (2002) 1.29
MucA-mediated coordination of type III secretion and alginate synthesis in Pseudomonas aeruginosa. J Bacteriol (2004) 1.29
Incidence, clinical predictors, genomics, and outcome of acute kidney injury among trauma patients. Ann Surg (2010) 1.27
Light whole genome sequence for SNP discovery across domestic cat breeds. BMC Genomics (2010) 1.27
Production of clinical-grade recombinant adeno-associated virus vectors. Curr Opin Biotechnol (2002) 1.26
An in vivo inducible gene of Pseudomonas aeruginosa encodes an anti-ExsA to suppress the type III secretion system. Mol Microbiol (2004) 1.26
In vivo complementation of complex I by the yeast Ndi1 enzyme. Possible application for treatment of Parkinson disease. J Biol Chem (2006) 1.25
Microarray analysis of A549 cells infected with rabbitpox virus (RPV): a comparison of wild-type RPV and RPV deleted for the host range gene, SPI-1. Virology (2003) 1.23
Interleukin 10 attenuates neointimal proliferation and inflammation in aortic allografts by a heme oxygenase-dependent pathway. Proc Natl Acad Sci U S A (2005) 1.22
IL-10 regulation of lupus in the NZM2410 murine model. Lab Invest (2006) 1.21
Severe injury is associated with insulin resistance, endoplasmic reticulum stress response, and unfolded protein response. Ann Surg (2012) 1.21
Role of Porphyromonas gingivalis SerB in gingival epithelial cell cytoskeletal remodeling and cytokine production. Infect Immun (2008) 1.21
Human Treg responses allow sustained recombinant adeno-associated virus-mediated transgene expression. J Clin Invest (2013) 1.20
Sirtuin 1 functionally and physically interacts with disruptor of telomeric silencing-1 to regulate alpha-ENaC transcription in collecting duct. J Biol Chem (2009) 1.20
Capsid serotype and timing of injection determines AAV transduction in the neonatal mice brain. PLoS One (2013) 1.19
A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS Gene therapy in Canavan mice. Mol Ther (2013) 1.19
Mutational and functional analysis reveals ADAMTS18 metalloproteinase as a novel driver in melanoma. Mol Cancer Res (2010) 1.19
Exome sequencing and SNP analysis detect novel compound heterozygosity in fatty acid hydroxylase-associated neurodegeneration. Eur J Hum Genet (2011) 1.18
Predisposition to cancer caused by genetic and functional defects of mammalian Atad5. PLoS Genet (2011) 1.18
Enhancing rAAV vector expression in the lung. J Gene Med (2005) 1.17
Efficient transduction of vascular endothelial cells with recombinant adeno-associated virus serotype 1 and 5 vectors. Hum Gene Ther (2005) 1.17
Ultrasensitive detection of biomolecules with fluorescent dye-doped nanoparticles. Anal Biochem (2004) 1.17
Translating the genomics revolution: the need for an international gene therapy consortium for monogenic diseases. Mol Ther (2013) 1.16
Functional characterization of a recombinant adeno-associated virus 5-pseudotyped cystic fibrosis transmembrane conductance regulator vector. Hum Gene Ther (2004) 1.16
Expression profiling after activation of amino acid deprivation response in HepG2 human hepatoma cells. Physiol Genomics (2010) 1.15
Changes in transcription within the CA1 field of the hippocampus are associated with age-related spatial learning impairments. Neurobiol Learn Mem (2006) 1.14
Patterns of gene expression reveal a temporally orchestrated wound healing response in the injured spinal cord. J Neurosci (2004) 1.13
Flagellin enhances NK cell proliferation and activation directly and through dendritic cell-NK cell interactions. J Leukoc Biol (2005) 1.12
Recurrent pneumonia in children: a case report and approach to diagnosis. Clin Pediatr (Phila) (2006) 1.12
Exome sequencing as a diagnostic tool in a case of undiagnosed juvenile-onset GM1-gangliosidosis. Neurology (2012) 1.11
Preclinical evaluation of a recombinant adeno-associated virus vector expressing human alpha-1 antitrypsin made using a recombinant herpes simplex virus production method. Hum Gene Ther (2010) 1.08
Efficient hepatic delivery and expression from a recombinant adeno-associated virus 8 pseudotyped alpha1-antitrypsin vector. Mol Ther (2005) 1.07
Sustained miRNA-mediated knockdown of mutant AAT with simultaneous augmentation of wild-type AAT has minimal effect on global liver miRNA profiles. Mol Ther (2012) 1.07
Specific inactivation of two immunomodulatory SIGLEC genes during human evolution. Proc Natl Acad Sci U S A (2012) 1.06
Persistent inflammation, immunosuppression, and catabolism syndrome after severe blunt trauma. J Trauma Acute Care Surg (2014) 1.06
Voluntary exercise prevents the obese and diabetic metabolic syndrome of the melanocortin-4 receptor knockout mouse. FASEB J (2008) 1.06
Genomic and proteomic determinants of outcome in patients undergoing thoracoabdominal aortic aneurysm repair. J Immunol (2004) 1.06
Prevalence of day-care centre children (France) with faecal CTX-M-producing Escherichia coli comprising O25b:H4 and O16:H5 ST131 strains. J Antimicrob Chemother (2014) 1.06
Soy isoflavones alter expression of genes associated with cancer progression, including interleukin-8, in androgen-independent PC-3 human prostate cancer cells. J Nutr (2006) 1.05
Therapeutic level of functional human alpha 1 antitrypsin (hAAT) secreted from murine muscle transduced by adeno-associated virus (rAAV1) vector. J Gene Med (2006) 1.04
Roles of vaccinia virus genes E3L and K3L and host genes PKR and RNase L during intratracheal infection of C57BL/6 mice. J Virol (2010) 1.04
Cystic fibrosis transmembrane conductance regulator deficiency exacerbates islet cell dysfunction after beta-cell injury. Diabetes (2006) 1.03
In vivo post-transcriptional gene silencing of alpha-1 antitrypsin by adeno-associated virus vectors expressing siRNA. Lab Invest (2007) 1.03
Impact on genetic networks in human macrophages by a CCR5 strain of human immunodeficiency virus type 1. J Virol (2004) 1.02
Lack of cystic fibrosis transmembrane conductance regulator in CD3+ lymphocytes leads to aberrant cytokine secretion and hyperinflammatory adaptive immune responses. Am J Respir Cell Mol Biol (2010) 1.02
Protective immunity and defects in the neonatal and elderly immune response to sepsis. J Immunol (2014) 1.01
Deposition and expression of aerosolized rAAV vectors in the lungs of Rhesus macaques. Mol Ther (2002) 1.00
Functional genomics and gene expression profiling in sepsis: beyond class prediction. Clin Infect Dis (2005) 1.00
Microfluidics-based capture of human neutrophils for expression analysis in blood and bronchoalveolar lavage. Lab Invest (2011) 1.00
Microfluidic leukocyte isolation for gene expression analysis in critically ill hospitalized patients. Clin Chem (2008) 0.99
A single-subunit NADH-quinone oxidoreductase renders resistance to mammalian nerve cells against complex I inhibition. Mol Ther (2002) 0.99