Published in Am J Respir Cell Mol Biol on March 01, 2014
Origins of cystic fibrosis lung disease. N Engl J Med (2015) 2.97
The innate immune function of airway epithelial cells in inflammatory lung disease. Eur Respir J (2015) 1.05
Directly sampling the lung of a young child with cystic fibrosis reveals diverse microbiota. Ann Am Thorac Soc (2014) 0.97
The draft genome sequence of the ferret (Mustela putorius furo) facilitates study of human respiratory disease. Nat Biotechnol (2014) 0.94
Defective innate immunity and hyperinflammation in newborn cystic fibrosis transmembrane conductance regulator-knockout ferret lungs. Am J Respir Cell Mol Biol (2015) 0.94
Animal models of gastrointestinal and liver diseases. Animal models of cystic fibrosis: gastrointestinal, pancreatic, and hepatobiliary disease and pathophysiology. Am J Physiol Gastrointest Liver Physiol (2015) 0.84
Ferret and pig models of cystic fibrosis: prospects and promise for gene therapy. Hum Gene Ther Clin Dev (2015) 0.84
Early pulmonary disease manifestations in cystic fibrosis mice. J Cyst Fibros (2016) 0.81
A ferret model of COPD-related chronic bronchitis. JCI Insight (2016) 0.81
Cystic fibrosis mouse model-dependent intestinal structure and gut microbiome. Mamm Genome (2015) 0.80
Pancreatic pathophysiology in cystic fibrosis. J Pathol (2015) 0.79
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
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
A Transient Metabolic Recovery from Early Life Glucose Intolerance in Cystic Fibrosis Ferrets Occurs During Pancreatic Remodeling. Endocrinology (2016) 0.76
Optimization of Recombinant Adeno-Associated Virus-Mediated Expression for Large Transgenes, Using a Synthetic Promoter and Tandem Array Enhancers. Hum Gene Ther (2015) 0.75
Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis. J Innate Immun (2016) 0.75
Stem cell-derived organoids to model gastrointestinal facets of cystic fibrosis. United European Gastroenterol J (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
Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. Cell (1998) 6.72
CFTR as a cAMP-dependent regulator of sodium channels. Science (1995) 6.50
Genome fingerprinting of Pseudomonas aeruginosa indicates colonization of cystic fibrosis siblings with closely related strains. J Clin Microbiol (1988) 4.27
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
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
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest (2010) 2.45
Normal mouse intestinal mucus release requires cystic fibrosis transmembrane regulator-dependent bicarbonate secretion. J Clin Invest (2009) 2.37
Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia. Cell (2010) 2.24
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
The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. Hum Pathol (1976) 2.09
Cystic fibrosis mouse models. Am J Respir Cell Mol Biol (2006) 1.98
Adeno-associated virus-targeted disruption of the CFTR gene in cloned ferrets. J Clin Invest (2008) 1.64
Mouse models of cystic fibrosis: phenotypic analysis and research applications. J Cyst Fibros (2011) 1.61
A new role for bicarbonate in mucus formation. Am J Physiol Lung Cell Mol Physiol (2010) 1.52
Abnormal endocrine pancreas function at birth in cystic fibrosis ferrets. J Clin Invest (2012) 1.40
Innate immunity in cystic fibrosis lung disease. J Cyst Fibros (2012) 1.38
Improved performance of bacterium and yeast identification by a commercial matrix-assisted laser desorption ionization-time of flight mass spectrometry system in the clinical microbiology laboratory. J Clin Microbiol (2011) 1.34
Comparing the microbiota of the cystic fibrosis lung and human gut. Gut Microbes (2010) 0.97
CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice. J Clin Invest (2011) 0.95
The cystic fibrosis airway microbiome. Curr Opin Pulm Med (2012) 0.89
Bioelectric characterization of epithelia from neonatal CFTR knockout ferrets. Am J Respir Cell Mol Biol (2013) 0.88
Comparative processing and function of human and ferret cystic fibrosis transmembrane conductance regulator. J Biol Chem (2012) 0.84
Anaplastic lymphoma kinase inhibition in non-small-cell lung cancer. N Engl J Med (2010) 36.78
Early palliative care for patients with metastatic non-small-cell lung cancer. N Engl J Med (2010) 29.06
Evolution and functional impact of rare coding variation from deep sequencing of human exomes. Science (2012) 17.12
Genotypic and histological evolution of lung cancers acquiring resistance to EGFR inhibitors. Sci Transl Med (2011) 16.94
Clinical features and outcome of patients with non-small-cell lung cancer who harbor EML4-ALK. J Clin Oncol (2009) 15.23
Detection of mutations in EGFR in circulating lung-cancer cells. N Engl J Med (2008) 13.54
Traces of human migrations in Helicobacter pylori populations. Science (2003) 11.92
First-line gefitinib in patients with advanced non-small-cell lung cancer harboring somatic EGFR mutations. J Clin Oncol (2008) 9.43
Irreversible inhibitors of the EGF receptor may circumvent acquired resistance to gefitinib. Proc Natl Acad Sci U S A (2005) 6.82
Rapid targeted mutational analysis of human tumours: a clinical platform to guide personalized cancer medicine. EMBO Mol Med (2010) 6.38
Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs. Science (2008) 6.28
A test of enhancing model accuracy in high-throughput crystallography. J Struct Funct Genomics (2005) 5.96
Molecular predictors of response to epidermal growth factor receptor antagonists in non-small-cell lung cancer. J Clin Oncol (2007) 5.55
Evidence for an epigenetic mechanism by which Hsp90 acts as a capacitor for morphological evolution. Nat Genet (2002) 5.37
Modular flexibility of dystrophin: implications for gene therapy of Duchenne muscular dystrophy. Nat Med (2002) 5.07
Deep resequencing reveals excess rare recent variants consistent with explosive population growth. Nat Commun (2010) 4.39
Sirt3-mediated deacetylation of evolutionarily conserved lysine 122 regulates MnSOD activity in response to stress. Mol Cell (2010) 4.19
Exon 19 deletion mutations of epidermal growth factor receptor are associated with prolonged survival in non-small cell lung cancer patients treated with gefitinib or erlotinib. Clin Cancer Res (2006) 4.11
Epidermal growth factor receptor mutations and gene amplification in non-small-cell lung cancer: molecular analysis of the IDEAL/INTACT gefitinib trials. J Clin Oncol (2005) 4.04
Systemic administration of optimized aptamer-siRNA chimeras promotes regression of PSMA-expressing tumors. Nat Biotechnol (2009) 3.78
Phase II clinical trial of chemotherapy-naive patients > or = 70 years of age treated with erlotinib for advanced non-small-cell lung cancer. J Clin Oncol (2007) 3.50
Neratinib, an irreversible pan-ErbB receptor tyrosine kinase inhibitor: results of a phase II trial in patients with advanced non-small-cell lung cancer. J Clin Oncol (2010) 3.40
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
Origins of cystic fibrosis lung disease. N Engl J Med (2015) 2.97
Longitudinal perceptions of prognosis and goals of therapy in patients with metastatic non-small-cell lung cancer: results of a randomized study of early palliative care. J Clin Oncol (2011) 2.95
XPD and XRCC1 genetic polymorphisms are prognostic factors in advanced non-small-cell lung cancer patients treated with platinum chemotherapy. J Clin Oncol (2004) 2.93
Distinguishing human ethnic groups by means of sequences from Helicobacter pylori: lessons from Ladakh. Proc Natl Acad Sci U S A (2004) 2.83
Analysis of potential predictive markers of cetuximab benefit in BMS099, a phase III study of cetuximab and first-line taxane/carboplatin in advanced non-small-cell lung cancer. J Clin Oncol (2010) 2.80
Ubiquitination of both adeno-associated virus type 2 and 5 capsid proteins affects the transduction efficiency of recombinant vectors. J Virol (2002) 2.77
Identification of a KCNE2 gain-of-function mutation in patients with familial atrial fibrillation. Am J Hum Genet (2004) 2.66
Whole-genome sequence-based analysis of high-density lipoprotein cholesterol. Nat Genet (2013) 2.62
MALAT-1: a long non-coding RNA and its important 3' end functional motif in colorectal cancer metastasis. Int J Oncol (2011) 2.59
A Kir2.1 gain-of-function mutation underlies familial atrial fibrillation. Biochem Biophys Res Commun (2005) 2.55
The porcine lung as a potential model for cystic fibrosis. Am J Physiol Lung Cell Mol Physiol (2008) 2.53
Circulating 25-hydroxyvitamin D levels predict survival in early-stage non-small-cell lung cancer patients. J Clin Oncol (2007) 2.52
Induction chemoradiotherapy followed by resection for locally advanced Masaoka stage III and IVA thymic tumors. Ann Thorac Surg (2008) 2.47
Disease phenotype of a ferret CFTR-knockout model of cystic fibrosis. J Clin Invest (2010) 2.45
SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. J Clin Invest (2008) 2.42
A phase II study of gefitinib in patients with advanced thyroid cancer. Thyroid (2008) 2.41
Genome sequencing and comparison of two nonhuman primate animal models, the cynomolgus and Chinese rhesus macaques. Nat Biotechnol (2011) 2.37
Timing of adjuvant chemotherapy initiation after surgery for stage III colon cancer. Cancer (2006) 2.36
Delay of adjuvant chemotherapy initiation following breast cancer surgery among elderly women. Breast Cancer Res Treat (2006) 2.26
Duration of adjuvant chemotherapy for colon cancer and survival among the elderly. J Clin Oncol (2006) 2.26
Wnt-3A/beta-catenin signaling induces transcription from the LEF-1 promoter. J Biol Chem (2002) 2.23
Exploring techniques for vision based human activity recognition: methods, systems, and evaluation. Sensors (Basel) (2013) 2.22
Synthesis of the H-cluster framework of iron-only hydrogenase. Nature (2005) 2.17
Efficient in vivo gene expression by trans-splicing adeno-associated viral vectors. Nat Biotechnol (2005) 2.16
The TCF-1 and LEF-1 transcription factors have cooperative and opposing roles in T cell development and malignancy. Immunity (2012) 2.15
Targeted injury of type II alveolar epithelial cells induces pulmonary fibrosis. Am J Respir Crit Care Med (2009) 2.15
Nox2 and Rac1 regulate H2O2-dependent recruitment of TRAF6 to endosomal interleukin-1 receptor complexes. Mol Cell Biol (2006) 2.12
T-cell immunoglobulin and mucin domain 1 (TIM-1) is a receptor for Zaire Ebolavirus and Lake Victoria Marburgvirus. Proc Natl Acad Sci U S A (2011) 2.12
Immune-related microRNAs are abundant in breast milk exosomes. Int J Biol Sci (2011) 2.11
Integration of molecular profiling into the lung cancer clinic. Clin Cancer Res (2009) 2.06
Role of extracellular superoxide dismutase in the mouse angiotensin slow pressor response. Hypertension (2006) 2.05
The ΔF508 mutation causes CFTR misprocessing and cystic fibrosis-like disease in pigs. Sci Transl Med (2011) 2.01
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
VEGF polymorphisms and survival in early-stage non-small-cell lung cancer. J Clin Oncol (2008) 1.94
Excision repair cross-complementation group 1 polymorphism predicts overall survival in advanced non-small cell lung cancer patients treated with platinum-based chemotherapy. Clin Cancer Res (2004) 1.91
Airway epithelial cells: current concepts and challenges. Proc Am Thorac Soc (2008) 1.90
Foxp3+ CD4 regulatory T cells limit pulmonary immunopathology by modulating the CD8 T cell response during respiratory syncytial virus infection. J Immunol (2010) 1.88
Phase II study of recombinant human endostatin in patients with advanced neuroendocrine tumors. J Clin Oncol (2006) 1.86
Gene therapy for adenosine deaminase-deficient severe combined immune deficiency: clinical comparison of retroviral vectors and treatment plans. Blood (2012) 1.85
Serum and urine metabolite profiling reveals potential biomarkers of human hepatocellular carcinoma. Mol Cell Proteomics (2011) 1.81
A phase I study with neratinib (HKI-272), an irreversible pan ErbB receptor tyrosine kinase inhibitor, in patients with solid tumors. Clin Cancer Res (2009) 1.79
Focal adhesion kinase is required for intestinal regeneration and tumorigenesis downstream of Wnt/c-Myc signaling. Dev Cell (2010) 1.79
Cell of origin strongly influences genetic selection in a mouse model of T-ALL. Blood (2011) 1.78
Delay in initiating adjuvant radiotherapy following breast conservation surgery and its impact on survival. Int J Radiat Oncol Biol Phys (2006) 1.77
Crystal structure of a Cbf5-Nop10-Gar1 complex and implications in RNA-guided pseudouridylation and dyskeratosis congenita. Mol Cell (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
Response to treatment and survival of patients with non-small cell lung cancer undergoing somatic EGFR mutation testing. Oncologist (2007) 1.75
Toward routine use of 3D histopathology as a research tool. Am J Pathol (2012) 1.73
EGFR mutation status and survival after diagnosis of brain metastasis in nonsmall cell lung cancer. Neuro Oncol (2010) 1.73
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
ABO incompatible renal transplantation: a paradigm ready for broad implementation. Transplantation (2009) 1.71
Phase I/II study of AT-101 with topotecan in relapsed and refractory small cell lung cancer. J Thorac Oncol (2010) 1.71