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
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Gene therapy strategies for idiopathic pulmonary fibrosis: recent advances, current challenges, and future directions.
TL;DR: In this paper, a review of the use of gene therapies to treat pulmonary fibrosis in animals is presented, with recommendations for future research to address challenges with respect to the selection and use of animal models as well as the development of delivery vectors and dosage forms.
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Quantitative assessment of radiation dose and fractionation effects on normal tissue by utilizing a novel lung fibrosis index model.
Cheng Zhou,Bleddyn Jones,Mahmoud Moustafa,Christian Schwager,Christian Schwager,Julia Bauer,Bing Yang,Liji Cao,Min Jia,Andrea Mairani,Ming Chen,Longhua Chen,Juergen Debus,Juergen Debus,Amir Abdollahi,Amir Abdollahi +15 more
TL;DR: The systematic radiobiological characterization of RILF in the C57BL/6 mouse reported in this study marks an important step towards precise estimation of dose-response for development of lung fibrosis.
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Low-dose administration of bleomycin leads to early alterations in lung mechanics
Lauren Headley,Lauren Headley,Weizhen Bi,Cory Wilson,Scott D. Collum,Melissa Chavez,Tamara Darwiche,Tinne C.J. Mertens,Adriana M. Hernandez,Saad R. Siddiqui,Stephanie Rosenbaum,Richard A. Johnston,Harry Karmouty-Quintana +12 more
TL;DR: Findings are significant to refine drug discovery in idiopathic pulmonary fibrosis, where preclinical studies using lung function parameters would enhance the translational potential of drug candidates where lung function readouts are routinely performed in the clinic.
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You Say You Want a Resolution (of Fibrosis).
TL;DR: Three critical areas of tissue homeostasis that are necessary for fibrosis resolution are emphasized, namely the elimination of matrix-producing cells, the clearance of excess matrix and the regeneration of normal tissue constituents.
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Density functional studies and spectroscopic analysis (FT-IR, FT-Raman, UV–visible, and NMR)with molecular docking approach on an antifibrotic drug Pirfenidone
TL;DR: In this article, the authors contributed to the findings of geometrical structure, vibrational frequencies and respective assignments with the compatible electronic, optical, nonlinear and thermodynamic properties of an antifibrotic drug.
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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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