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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Journal ArticleDOI
Effect of Hypoxia on Pulmonary Endothelial Cells from Bleomycin-Induced Pulmonary Fibrosis Model Mice
Daisuke Akahori,Naoki Inui,Yusuke Inoue,Hideki Yasui,Hironao Hozumi,Yuzo Suzuki,Masato Karayama,Kazuki Furuhashi,Noriyuki Enomoto,Tomoyuki Fujisawa,Takafumi Suda +10 more
TL;DR: Levels of plasminogen activator inhibitor 1, von Willebrand factor, and matrix metalloproteinase 12 were increased in endothelial cells isolated from bleomycin-treated mice exposed to hypoxic conditions, and levels of fibrotic mediators, transforming growth factor-β and connective tissue growth factor, were elevated only in endothelium cells from bleomcin- treated and not from saline-treated lungs.
Journal ArticleDOI
Bleomycin‐induced lung injury: Revisiting an old tool to model group III PH associated with pulmonary fibrosis
D. Santos-Ribeiro,Marylène Lecocq,M M De Beukelaer,Caroline Bouzin,Mihaly Palmai-Pallag,Yousef Yakoub,François Huaux,Sandrine Horman,Frédéric Perros,Charles Pilette,Laurent Godinas +10 more
TL;DR: In this paper , a rat model of group III pulmonary hypertension associated with interstitial lung diseases such as pulmonary fibrosis (PF) was validated by using bleomycin (BM) intratracheal instillation and characterizing the nature of induced lung and vascular remodeling, including the influence on RV structure and function.
Journal ArticleDOI
Systemic mesalazine treatment prevents spontaneous skin fibrosis in PLK2-deficient mice
Manja Newe,Theresa A Kant,Maximilian Hoffmann,Johanna S E Rausch,Luise Winter,Karolina Künzel,Erik Klapproth,Claudia Günther,Stephan R Künzel +8 more
TL;DR: In this paper, the role of polo-like kinase 2 (PLK2) and the pro-fibrotic cytokine osteopontin (OPN) in the pathogenesis of cutaneous fibrosis was investigated.
Fibrotic Human Lung Extracellular Matrix as a Disease- Specific Substrate for Models of Pulmonary Fibrosis
Igal Germanguz,Evelyn Aranda,Jennifer C Xiong,Natalia Kissel,Alexandra Nichols,Eddie Gadee,John D. O’Neill +6 more
TL;DR: The development and characterization of a human pulmonary fibrosisspecific cell culture substrate comprised of intact fibrotic lung extracellular matrix that recapitulates the human IPF disease environment in vitro and the applicability is demonstrated for 3D in-vitro models of IPF and cell-based assays in early-stage drug discovery.
Journal ArticleDOI
Immune Mechanisms of Pulmonary Fibrosis with Bleomycin
TL;DR: In this article , the authors examined the common mechanisms of lung wound-healing responses after BLM-induced lung injury as well as the pathogenesis of the most common pulmonary fibrosis.
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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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