Animal models of fibrotic lung disease.
Bethany B. Moore,William Lawson,William Lawson,Tim D. Oury,Thomas H. Sisson,Krishnan Raghavendran,Cory M. Hogaboam +6 more
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TLDR
Each of the models reviewed in this report offers a powerful tool for studying some aspect of fibrotic lung disease and has a much better understanding of the fact that the aged lung has increased susceptibility to fibrosis.Abstract:
Interstitial lung fibrosis can develop as a consequence of occupational or medical exposure, as a result of genetic defects, and after trauma or acute lung injury leading to fibroproliferative acute respiratory distress syndrome, or it can develop in an idiopathic manner. The pathogenesis of each form of lung fibrosis remains poorly understood. They each result in a progressive loss of lung function with increasing dyspnea, and most forms ultimately result in mortality. To better understand the pathogenesis of lung fibrotic disorders, multiple animal models have been developed. This review summarizes the common and emerging models of lung fibrosis to highlight their usefulness in understanding the cell–cell and soluble mediator interactions that drive fibrotic responses. Recent advances have allowed for the development of models to study targeted injuries of Type II alveolar epithelial cells, fibroblastic autonomous effects, and targeted genetic defects. Repetitive dosing in some models has more closely mimicked the pathology of human fibrotic lung disease. We also have a much better understanding of the fact that the aged lung has increased susceptibility to fibrosis. Each of the models reviewed in this report offers a powerful tool for studying some aspect of fibrotic lung disease.read more
Citations
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Development of experimental silicosis in inbred and outbred mice depends on instillation volume
TL;DR: The critical importance of instillation volume on the induction, severity, and type of inflammatory pathology in experimental silicosis is underscored.
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Dysfunctional lactate metabolism in human alveolar type II cells from idiopathic pulmonary fibrosis lung explant tissue.
TL;DR: In this article, the alveolar type II epithelial cell (AEC2) was isolated from human patient specimens of non-fibrotic and IPF lungs were used for metabolic measurements, lactate dehydrogenase (LDH) analyses and siRNA-mediated knockdown experiments.
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Glucose metabolism in idiopathic pulmonary fibrosis
TL;DR: The data presented in this thesis demonstrate that the metabolic phenotype of fibroblasts changes during TGFβ1-induced fibroblast differentiation and is regulated by mTOR, in a PI3K-AKT-independent manner.
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Inhibition of chlorine-induced airway fibrosis by budesonide.
Sadiatu Musah,Jing Chen,Connie F. Schlueter,David M. Humphrey,Kendall Stocke,Mona I. Hoyle,Gary W. Hoyle +6 more
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Keratin14 mRNA expression in human pneumocytes during quiescence, repair and disease
Marco Confalonieri,Emanuele Buratti,Gabriele Grassi,Rossana Bussani,Marco Chilosi,Rossella Farra,Michela Abrami,Cristiana Stuani,Francesco Salton,Miriam Ficial,Paola Confalonieri,Lorenzo Zandonà,Maurizio Romano,Maurizio Romano +13 more
TL;DR: The results suggest that K RT14 is a viable biomarker of pneumocyte activation, and repair/regeneration, and the involvement of KRT14 in regenerative process may suggest a novel pharmaceutical target to accelerate lung repair.
References
More filters
Journal ArticleDOI
Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome.
Roy G. Brower,Michael A. Matthay,Alan H. Morris,David A. Schoenfeld,B. Taylor Thompson,Arthur P. Wheeler +5 more
TL;DR: In patients with acute lung injury and the acute respiratory distress syndrome, mechanical ventilation with a lower tidal volume than is traditionally used results in decreased mortality and increases the number of days without ventilator use.
Journal ArticleDOI
American thoracic society/European respiratory society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias
William D. Travis,Talmadge E. King,Eric D. Bateman,David A. Lynch,Frédrique Capron,Thomas V. Colby,Jean-François Cordier,Roland M. Dubois,Jeffrey R. Galvin,Philippe Grenier,David M. Hansell,Gary W. Hunninghake,Masanori Kitaichi,Nestor L. Müller,Jeffrey L. Myers,Sonoko Nagai,Andrew G. Nicholson,Ganesh Raghu,Benoit Wallaert,Christian Brambilla,Kevin K. Brown,Andrew L. Cherniaev,Ulrich Costabel,David B. Coultas,Gerald S. Davis,Maurits G. Demedts,William W. Douglas,Jim J. Egan,Anders Eklund,Leonarda M. Fabbri,Craig A. Henke,Richard Hubbard,Y. Inoue,Takateru Izumi,H. M. Jansen,Ian Johnston,Dong Soon Kim,Nasreen Khalil,Fiona R. Lake,Giuseppe Lungarella,Joseph P. Lynch,Douglas W. Mapel,Fernando J. Martinez,Richard A. Matthay,Lee S. Newman,Paul W. Noble,Ken Ohta,Dario Olivieri,Luis A. Ortiz,Venerino Poletti,Robert Rodriguez-Roisin,William N. Rom,Jay Hoon Ryu,Paulo Hilário Nascimento Saldiva,Raúl H Sansores,Marvin L. Schwarz,Moisés Selman,Cecelia M. Smith,Zhaohui Tong,Zarir F Udwadia,Dominique Valeyre,Athol U. Wells,Robert A. Wise,Antonio Xaubet,Emilio Alvarez Fernandez,Elisabeth Brambilla,Vera Luiza Capelozzi,Andrew Cherniaev,Peter Dalquen,Gerhard Dekan,Philip S. Hasleton,James C. Hogg,N. A. Jambhekar,Anna Luise A Katzenstein,Michael Koss,Osamu Matsubara,Klaus Michael Müller,F. B.J.M. Thunnissen,James A. Waldron,Wei Hua Li,Paul J. Friedman,Martin Remy-Jardin,Theresa C. McLoud +82 more
TL;DR: The Diagnostic Process Is Dynamic Clinical Evaluation Radiological Evaluation Role of Surgical Lung Biopsy Unclassifiable Interstitial Pneumonia Bronchoalveolar Lavage Fluid Evaluation Idiopathic Pulmonary Fibrosis.
Journal ArticleDOI
Idiopathic pulmonary fibrosis : Diagnosis and treatment. International consensus statement
Jr King,Ulrich Costabel,Jean-François Cordier,G. A. DoPico,R. M. DuBois,David A. Lynch,Joseph P. Lynch,J.L. Myers,R. Panos,Ganesh Raghu,David A. Schwartz,C. M. Smith +11 more
TL;DR: Many acute and chronic lung disorders with variable degrees of pulmonary inflammation and fibrosis are collectively referred to as interstitial lung diseases (ILDs) or diffuse parenchymal lung diseases.
Journal ArticleDOI
Humanized mice in translational biomedical research
TL;DR: This Review discusses the development of these new generations of humanized mice, how they will facilitate translational research in several biomedical disciplines and approaches to overcome the remaining limitations of these models.
Journal ArticleDOI
Telomerase Mutations in Families with Idiopathic Pulmonary Fibrosis
Mary Armanios,Julian J.-L. Chen,Joy D. Cogan,Jonathan K. Alder,Roxann G. Ingersoll,Cheryl Markin,William Lawson,William Lawson,Mingyi Xie,Irma Vulto,John A. Phillips,Peter M. Lansdorp,Carol W. Greider,James E. Loyd +13 more
TL;DR: In this paper, the authors found that mutations in the genes encoding telomerase components can appear as familial idiopathic pulmonary fibrosis, which is a rare hereditary disorder associated with premature death from aplastic anemia and lung fibrosis.
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