Search Results - "Wert, Susan"
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Diseases of Pulmonary Surfactant Homeostasis
Published in Annual review of pathology (24-01-2015)“…Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm…”
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Alveolar surfactant homeostasis and the pathogenesis of pulmonary disease
Published in Annual review of medicine (01-01-2010)“…The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after…”
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3
Sox2 is required for maintenance and differentiation of bronchiolar Clara, ciliated, and goblet cells
Published in PloS one (14-12-2009)“…The bronchioles of the murine lung are lined by a simple columnar epithelium composed of ciliated, Clara, and goblet cells that together mediate barrier…”
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4
SPDEF is required for mouse pulmonary goblet cell differentiation and regulates a network of genes associated with mucus production
Published in The Journal of clinical investigation (01-10-2009)“…Various acute and chronic inflammatory stimuli increase the number and activity of pulmonary mucus-producing goblet cells, and goblet cell hyperplasia and…”
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5
Genotype-Phenotype Correlations for Infants and Children with ABCA3 Deficiency
Published in American journal of respiratory and critical care medicine (15-06-2014)“…Recessive mutations in the ATP-binding cassette transporter A3 (ABCA3) cause lethal neonatal respiratory failure and childhood interstitial lung disease. Most…”
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6
Heterogeneous Pulmonary Phenotypes Associated With Mutations in the Thyroid Transcription Factor Gene NKX2-1
Published in Chest (01-09-2013)“…Background Mutations in the gene encoding thyroid transcription factor, NKX2-1 , result in neurologic abnormalities, hypothyroidism, and neonatal respiratory…”
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Genetic Disorders of Surfactant Dysfunction
Published in Pediatric and developmental pathology (01-07-2009)“…Mutations in the genes encoding the surfactant proteins B and C (SP-B and SP-C) and the phospholipid transporter, ABCA3, are associated with respiratory…”
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Transcriptional programs controlling perinatal lung maturation
Published in PloS one (20-08-2012)“…The timing of lung maturation is controlled precisely by complex genetic and cellular programs. Lung immaturity following preterm birth frequently results in…”
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9
Human GM-CSF Autoantibodies and Reproduction of Pulmonary Alveolar Proteinosis
Published in The New England journal of medicine (31-12-2009)“…To the Editor: Idiopathic pulmonary alveolar proteinosis is a rare disease in which surfactant lipids and proteins accumulate in pulmonary alveolar macrophages…”
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10
Sox2 activates cell proliferation and differentiation in the respiratory epithelium
Published in American journal of respiratory cell and molecular biology (01-07-2011)“…Sox2, a transcription factor critical for the maintenance of embryonic stem cells and induction of pluripotent stem cells, is expressed exclusively in the…”
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11
Familial pulmonary alveolar proteinosis caused by mutations in CSF2RA
Published in The Journal of experimental medicine (24-11-2008)“…Primary pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by accumulation of surfactant in the lungs that is presumed to be mediated by…”
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Genome-Wide Transcriptional Profiling Reveals Connective Tissue Mast Cell Accumulation in Bronchopulmonary Dysplasia
Published in American journal of respiratory and critical care medicine (15-08-2012)“…Bronchopulmonary dysplasia (BPD) is a major complication of premature birth. Risk factors for BPD are complex and include prenatal infection and O(2) toxicity…”
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Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis
Published in Thorax (01-08-2011)“…Bronchiolisation of distal airspaces is an unexplained feature of idiopathic pulmonary fibrosis (IPF). The authors sought to identify mechanisms driving the…”
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14
R-spondin 2 is required for normal laryngeal-tracheal, lung and limb morphogenesis
Published in Development (Cambridge) (15-03-2008)“…Herein, we demonstrate that Lrp6-mediated R-spondin 2 signaling through the canonical Wnt pathway is required for normal morphogenesis of the respiratory tract…”
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15
Ontology-guided segmentation and object identification for developmental mouse lung immunofluorescent images
Published in BMC bioinformatics (23-02-2021)“…Immunofluorescent confocal microscopy uses labeled antibodies as probes against specific macromolecules to discriminate between multiple cell types. For images…”
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Sox17 is required for normal pulmonary vascular morphogenesis
Published in Developmental biology (01-03-2014)“…The SRY-box containing transcription factor Sox17 is required for endoderm formation and vascular morphogenesis during embryonic development. In the lung,…”
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Hereditary Pulmonary Alveolar Proteinosis: Pathogenesis, Presentation, Diagnosis, and Therapy
Published in American journal of respiratory and critical care medicine (15-11-2010)“…We identified a 6-year-old girl with pulmonary alveolar proteinosis (PAP), impaired granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor…”
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Comprehensive anatomic ontologies for lung development: A comparison of alveolar formation and maturation within mouse and human lung
Published in Journal of biomedical semantics (24-10-2019)“…Although the mouse is widely used to model human lung development, function, and disease, our understanding of the molecular mechanisms involved in…”
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Diffuse Lung Disease in Young Children: Application of a Novel Classification Scheme
Published in American journal of respiratory and critical care medicine (01-12-2007)“…Considerable confusion exists regarding nomenclature, classification, and management of pediatric diffuse lung diseases due to the relative rarity and…”
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ABCA3 Gene Mutations in Newborns with Fatal Surfactant Deficiency
Published in The New England journal of medicine (25-03-2004)“…This study describes the genetic cause of some cases of fatal surfactant deficiency in newborns. The gene encoding ATP-binding cassette transporter A3 ( ABCA3…”
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