Published in Am J Respir Cell Mol Biol on June 01, 2015
An unexpected effect of TNF-α on F508del-CFTR maturation and function. F1000Res (2015) 0.98
Claudins: Gatekeepers of lung epithelial function. Semin Cell Dev Biol (2015) 0.94
Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy. Hum Gene Ther Clin Dev (2015) 0.84
Newborn Cystic Fibrosis Pigs Have a Blunted Early Response to an Inflammatory Stimulus. Am J Respir Crit Care Med (2016) 0.81
Quantitative proteomics reveals an altered cystic fibrosis in vitro bronchial epithelial secretome. Am J Respir Cell Mol Biol (2015) 0.81
In vivo imaging of airway cilia and mucus clearance with micro-optical coherence tomography. Biomed Opt Express (2016) 0.81
Hypoxic epithelial necrosis triggers neutrophilic inflammation via IL-1 receptor signaling in cystic fibrosis lung disease. Am J Respir Crit Care Med (2015) 0.79
Pancreatic pathophysiology in cystic fibrosis. J Pathol (2015) 0.79
Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense. Am J Physiol Lung Cell Mol Physiol (2016) 0.78
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy. Am J Physiol Lung Cell Mol Physiol (2016) 0.77
Glandular Proteome Identifies Antiprotease Cystatin C as a Critical Modulator of Airway Hydration and Clearance. Am J Respir Cell Mol Biol (2016) 0.77
Animal Models of Cystic Fibrosis Pathology: Phenotypic Parallels and Divergences. Biomed Res Int (2016) 0.76
Galactomannan and Zymosan Block the Epinephrine-Induced Particle Transport in Tracheal Epithelium. PLoS One (2015) 0.75
Airway Microbiota in Bronchoalveolar Lavage Fluid from Clinically Well Infants with Cystic Fibrosis. PLoS One (2016) 0.75
Human Cystic Fibrosis Macrophages Have Defective Calcium-Dependent Protein Kinase C Activation of the NADPH Oxidase, an Effect Augmented by Burkholderia cenocepacia. J Immunol (2017) 0.75
Epithelial Anion Transport as Modulator of Chemokine Signaling. Mediators Inflamm (2016) 0.75
New use for an old drug: COX-independent anti-inflammatory effects of sulindac in models of cystic fibrosis. Br J Pharmacol (2016) 0.75
Cystic fibrosis. N Engl J Med (2005) 8.09
Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. Cell (1996) 6.84
Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest (2002) 5.94
Production of beta-defensins by human airway epithelia. Proc Natl Acad Sci U S A (1998) 3.85
CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. Nat Cell Biol (2006) 3.60
Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth. Sci Transl Med (2010) 3.36
Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest (2008) 3.33
Phosphorylation of the alpha subunit of eukaryotic initiation factor 2 is required for activation of NF-kappaB in response to diverse cellular stresses. Mol Cell Biol (2003) 3.01
Reduced airway surface pH impairs bacterial killing in the porcine cystic fibrosis lung. Nature (2012) 2.94
Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med (2007) 2.85
A novel host defense system of airways is defective in cystic fibrosis. Am J Respir Crit Care Med (2006) 2.61
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest (2010) 2.45
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell (2010) 2.24
Evidence for airway surface dehydration as the initiating event in CF airway disease. J Intern Med (2007) 2.23
Innate immunity in the respiratory epithelium. Am J Respir Cell Mol Biol (2011) 2.04
Cystic fibrosis: a mucosal immunodeficiency syndrome. Nat Med (2012) 2.03
Toll-like receptors in normal and cystic fibrosis airway epithelial cells. Am J Respir Cell Mol Biol (2003) 2.00
A physical linkage between cystic fibrosis airway surface dehydration and Pseudomonas aeruginosa biofilms. Proc Natl Acad Sci U S A (2006) 1.75
Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets. J Clin Invest (2008) 1.64
Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography. PLoS One (2013) 1.56
CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis. Biochemistry (2006) 1.54
CFTR involvement in chloride, bicarbonate, and liquid secretion by airway submucosal glands. Am J Physiol (1999) 1.54
Sputum biomarkers of inflammation in cystic fibrosis lung disease. Proc Am Thorac Soc (2007) 1.49
Lysozyme secretion by submucosal glands protects the airway from bacterial infection. Am J Respir Cell Mol Biol (2005) 1.49
Innate immunity in cystic fibrosis lung disease. J Cyst Fibros (2012) 1.38
Disease-causing mutations in the cystic fibrosis transmembrane conductance regulator determine the functional responses of alveolar macrophages. J Biol Chem (2009) 1.37
Innate immune responses to Pseudomonas aeruginosa infection. Microbes Infect (2011) 1.33
Role of the cystic fibrosis transmembrane conductance regulator in innate immunity to Pseudomonas aeruginosa infections. Proc Natl Acad Sci U S A (2000) 1.26
Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis. J Infect Dis (2004) 1.25
The alternative activation pathway and complement component C3 are critical for a protective immune response against Pseudomonas aeruginosa in a murine model of pneumonia. Infect Immun (2004) 1.21
Cystic fibrosis transmembrane conductance regulator-dependent regulation of epithelial inducible nitric oxide synthase expression. Am J Respir Cell Mol Biol (2000) 1.18
Salt-independent abnormality of antimicrobial activity in cystic fibrosis airway surface fluid. Am J Respir Cell Mol Biol (2001) 1.14
Effects of liver X receptor agonist treatment on pulmonary inflammation and host defense. J Immunol (2008) 1.11
Immune responses in cystic fibrosis: are they intrinsically defective? Am J Respir Cell Mol Biol (2012) 1.06
Association of lower airway inflammation with physiologic findings in young children with cystic fibrosis. Pediatr Pulmonol (2009) 1.06
Inhibition of mammalian target of rapamycin augments lipopolysaccharide-induced lung injury and apoptosis. J Immunol (2012) 1.04
Postnatal development of tracheal surface epithelium and submucosal glands in the ferret. Exp Lung Res (1986) 1.04
New animal models of cystic fibrosis: what are they teaching us? Curr Opin Pulm Med (2011) 1.03
Mapping the lung proteome in cystic fibrosis. J Proteome Res (2009) 1.01
Evolution of pulmonary inflammation and nutritional status in infants and young children with cystic fibrosis. Thorax (2011) 1.00
Lung phenotype of juvenile and adult cystic fibrosis transmembrane conductance regulator-knockout ferrets. Am J Respir Cell Mol Biol (2014) 0.97
Novel role for the liver X nuclear receptor in the suppression of lung inflammatory responses. J Biol Chem (2007) 0.97
Eukaryotic initiation factor 2 (eIF2) signaling regulates proinflammatory cytokine expression and bacterial invasion. J Biol Chem (2012) 0.97
Optimal complement-mediated phagocytosis of Pseudomonas aeruginosa by monocytes is cystic fibrosis transmembrane conductance regulator-dependent. Am J Respir Cell Mol Biol (2013) 0.90
Specific cleavage of the lung surfactant protein A by human cathepsin S may impair its antibacterial properties. Int J Biochem Cell Biol (2013) 0.89
Alterations in immune response and PPAR/LXR regulation in cystic fibrosis macrophages. J Cyst Fibros (2007) 0.89
Bioelectric characterization of epithelia from neonatal CFTR knockout ferrets. Am J Respir Cell Mol Biol (2013) 0.88
Low levels of insulin-like growth factor-1 contribute to alveolar macrophage dysfunction in cystic fibrosis. J Immunol (2013) 0.87
Cystic fibrosis lung disease: the role of nitric oxide. Pediatr Pulmonol (1999) 0.86
Airway defense mechanisms. Clin Chest Med (2012) 0.83
Error-correcting barcoded primers for pyrosequencing hundreds of samples in multiplex. Nat Methods (2008) 32.71
Improved signal-to-noise ratio in spectral-domain compared with time-domain optical coherence tomography. Opt Lett (2003) 10.17
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med (2011) 8.73
Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation. N Engl J Med (2010) 6.23
A test of enhancing model accuracy in high-throughput crystallography. J Struct Funct Genomics (2005) 5.96
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR. N Engl J Med (2015) 5.51
Three-dimensional microscopy of the tumor microenvironment in vivo using optical frequency domain imaging. Nat Med (2009) 5.41
Comprehensive volumetric optical microscopy in vivo. Nat Med (2006) 5.19
Modular flexibility of dystrophin: implications for gene therapy of Duchenne muscular dystrophy. Nat Med (2002) 5.07
Characterization of human atherosclerosis by optical coherence tomography. Circulation (2002) 5.04
Visualization of coronary atherosclerotic plaques in patients using optical coherence tomography: comparison with intravascular ultrasound. J Am Coll Cardiol (2002) 4.94
Unexpected diversity and complexity of the Guerrero Negro hypersaline microbial mat. Appl Environ Microbiol (2006) 4.74
In vivo human retinal imaging by ultrahigh-speed spectral domain optical coherence tomography. Opt Lett (2004) 4.43
Spectral domain optical coherence tomography: ultra-high speed, ultra-high resolution ophthalmic imaging. Arch Ophthalmol (2005) 3.56
Opportunistic pathogens enriched in showerhead biofilms. Proc Natl Acad Sci U S A (2009) 3.35
Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer. J Clin Invest (2008) 3.33
Optical coherence tomography to identify intramucosal carcinoma and high-grade dysplasia in Barrett's esophagus. Clin Gastroenterol Hepatol (2006) 3.28
In vivo characterization of coronary atherosclerotic plaque by use of optical coherence tomography. Circulation (2005) 3.23
Comprehensive microscopy of the esophagus in human patients with optical frequency domain imaging. Gastrointest Endosc (2008) 2.94
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
Ubiquitination of both adeno-associated virus type 2 and 5 capsid proteins affects the transduction efficiency of recombinant vectors. J Virol (2002) 2.77
Imaging the subcellular structure of human coronary atherosclerosis using micro-optical coherence tomography. Nat Med (2011) 2.60
A novel proteolytic cascade generates an extracellular matrix-derived chemoattractant in chronic neutrophilic inflammation. J Immunol (2008) 2.58
A critical role for LTA4H in limiting chronic pulmonary neutrophilic inflammation. Science (2010) 2.56
The porcine lung as a potential model for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol (2008) 2.53
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest (2010) 2.45
Intra-arterial catheter for simultaneous microstructural and molecular imaging in vivo. Nat Med (2011) 2.44
SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest (2008) 2.42
Tethered capsule endomicroscopy enables less invasive imaging of gastrointestinal tract microstructure. Nat Med (2013) 2.32
Identifying intestinal metaplasia at the squamocolumnar junction by using optical coherence tomography. Gastrointest Endosc (2006) 2.31
Comprehensive esophageal microscopy by using optical frequency-domain imaging (with video). Gastrointest Endosc (2007) 2.26
Wnt-3A/beta-catenin signaling induces transcription from the LEF-1 promoter. J Biol Chem (2002) 2.23
Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors. Nat Biotechnol (2005) 2.16
Targeted injury of type II alveolar epithelial cells induces pulmonary fibrosis. Am J Respir Crit Care Med (2009) 2.15
Millimeter-scale genetic gradients and community-level molecular convergence in a hypersaline microbial mat. Mol Syst Biol (2008) 2.12
Nox2 and Rac1 regulate H2O2-dependent recruitment of TRAF6 to endosomal interleukin-1 receptor complexes. Mol Cell Biol (2006) 2.12
Focal and multi-focal plaque macrophage distributions in patients with acute and stable presentations of coronary artery disease. J Am Coll Cardiol (2004) 2.03
Requirement for Rac1-dependent NADPH oxidase in the cardiovascular and dipsogenic actions of angiotensin II in the brain. Circ Res (2004) 1.98
Partial correction of endogenous DeltaF508 CFTR in human cystic fibrosis airway epithelia by spliceosome-mediated RNA trans-splicing. Nat Biotechnol (2002) 1.96
Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children. Am J Respir Crit Care Med (2010) 1.94
Cigarette smoke induces systemic defects in cystic fibrosis transmembrane conductance regulator function. Am J Respir Crit Care Med (2013) 1.93
Phase-gradient microscopy in thick tissue with oblique back-illumination. Nat Methods (2012) 1.92
Airway epithelial cells: current concepts and challenges. Proc Am Thorac Soc (2008) 1.90
Detection of high-risk atherosclerotic plaque: report of the NHLBI Working Group on current status and future directions. JACC Cardiovasc Imaging (2012) 1.85
A functional anatomic defect of the cystic fibrosis airway. Am J Respir Crit Care Med (2014) 1.85
Measurement of collagen and smooth muscle cell content in atherosclerotic plaques using polarization-sensitive optical coherence tomography. J Am Coll Cardiol (2007) 1.82
Image-guided biopsy in the esophagus through comprehensive optical frequency domain imaging and laser marking: a study in living swine. Gastrointest Endosc (2009) 1.79
Diagnostic accuracy of optical coherence tomography and integrated backscatter intravascular ultrasound images for tissue characterization of human coronary plaques. J Am Coll Cardiol (2006) 1.77
Virus-mediated transduction of murine retina with adeno-associated virus: effects of viral capsid and genome size. J Virol (2002) 1.77
Speckle reduction in optical coherence tomography images using digital filtering. J Opt Soc Am A Opt Image Sci Vis (2007) 1.75
>400 kHz repetition rate wavelength-swept laser and application to high-speed optical frequency domain imaging. Opt Lett (2010) 1.74
Atherosclerotic tissue characterization in vivo by optical coherence tomography attenuation imaging. J Biomed Opt (2010) 1.72
Bile acid regulation of C/EBPbeta, CREB, and c-Jun function, via the extracellular signal-regulated kinase and c-Jun NH2-terminal kinase pathways, modulates the apoptotic response of hepatocytes. Mol Cell Biol (2003) 1.72
In vivo association between positive coronary artery remodelling and coronary plaque characteristics assessed by intravascular optical coherence tomography. Eur Heart J (2008) 1.69
Volumetric optical frequency domain imaging of pulmonary pathology with precise correlation to histopathology. Chest (2013) 1.68
Molecular imaging of fibrin deposition in deep vein thrombosis using fibrin-targeted near-infrared fluorescence. JACC Cardiovasc Imaging (2012) 1.66
Optical coherence tomography as a tool for percutaneous coronary interventions. Catheter Cardiovasc Interv (2005) 1.66
Characterization of atherosclerotic plaques by laser speckle imaging. Circulation (2005) 1.66
A pharmacologic approach to acquired cystic fibrosis transmembrane conductance regulator dysfunction in smoking related lung disease. PLoS One (2012) 1.64
Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets. J Clin Invest (2008) 1.64
Phylogenetic diversity and ecology of environmental Archaea. Curr Opin Microbiol (2005) 1.62
Second-generation optical coherence tomography in clinical practice. High-speed data acquisition is highly reproducible in patients undergoing percutaneous coronary intervention. Rev Esp Cardiol (2010) 1.58
PITX2, beta-catenin and LEF-1 interact to synergistically regulate the LEF-1 promoter. J Cell Sci (2005) 1.58
Nox2-containing NADPH oxidase and Akt activation play a key role in angiotensin II-induced cardiomyocyte hypertrophy. Physiol Genomics (2006) 1.57
Wide-field fluorescence sectioning with hybrid speckle and uniform-illumination microscopy. Opt Lett (2008) 1.56
Method for quantitative study of airway functional microanatomy using micro-optical coherence tomography. PLoS One (2013) 1.56
Optically sectioned fluorescence endomicroscopy with hybrid-illumination imaging through a flexible fiber bundle. J Biomed Opt (2009) 1.55
Statistical properties of phase-decorrelation in phase-resolved Doppler optical coherence tomography. IEEE Trans Med Imaging (2009) 1.52
Signaling components of redox active endosomes: the redoxosomes. Antioxid Redox Signal (2009) 1.50
Lysozyme secretion by submucosal glands protects the airway from bacterial infection. Am J Respir Cell Mol Biol (2005) 1.49
Technology Insight: optical coherence tomography--current status and future development. Nat Clin Pract Cardiovasc Med (2006) 1.47
Redox modifier genes in amyotrophic lateral sclerosis in mice. J Clin Invest (2007) 1.46
Inflammation and airway microbiota during cystic fibrosis pulmonary exacerbations. PLoS One (2013) 1.43
Establishment of a reverse genetics system for studying human bocavirus in human airway epithelia. PLoS Pathog (2012) 1.43
Intravascular optical imaging technology for investigating the coronary artery. JACC Cardiovasc Imaging (2011) 1.42
Conformational change in coronary artery structure assessed by optical coherence tomography in patients with vasospastic angina. J Am Coll Cardiol (2011) 1.41
Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets. J Clin Invest (2012) 1.40
Expert review document part 2: methodology, terminology and clinical applications of optical coherence tomography for the assessment of interventional procedures. Eur Heart J (2012) 1.38
Tyrosine phosphorylation of I kappa B alpha activates NF kappa B through a redox-regulated and c-Src-dependent mechanism following hypoxia/reoxygenation. J Biol Chem (2002) 1.38
Explicet: graphical user interface software for metadata-driven management, analysis and visualization of microbiome data. Bioinformatics (2013) 1.38
Reliability of quantitative real-time PCR for bacterial detection in cystic fibrosis airway specimens. PLoS One (2010) 1.37
The effects of airway microbiome on corticosteroid responsiveness in asthma. Am J Respir Crit Care Med (2013) 1.36
Measurement of fibrous cap thickness in atherosclerotic plaques by spatiotemporal analysis of laser speckle images. J Biomed Opt (2006) 1.36
Intravascular laser speckle imaging catheter for the mechanical evaluation of the arterial wall. J Biomed Opt (2011) 1.35
Measuring and improving respiratory outcomes in cystic fibrosis lung disease: opportunities and challenges to therapy. J Cyst Fibros (2009) 1.35