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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The long pentraxin PTX3 promotes fibrocyte differentiation.
TL;DR: Data suggest that the relative levels of SAP and PTX3 present at sites of fibrosis may have a significant effect on the ability of monocytes to differentiate into fibrocytes.
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HDAC8 inhibition ameliorates pulmonary fibrosis
Shigeki Saito,Yan Zhuang,Takayoshi Suzuki,Yosuke Ota,Marjorie E. Bateman,Ala Alkhatib,Gilbert F. Morris,Joseph A. Lasky +7 more
TL;DR: It is observed that HDAC8 expression was increased in IPF lung tissue as well as transforming growth factor (TGF)β1-treated normal human lung fibroblasts (NHLFs) and that there is a therapeutic potential forHDAC8 inhibitors to treat IPF as wellas other fibrotic lung diseases.
Journal ArticleDOI
Translational models of lung disease.
TL;DR: A review of initiatives to identify state-of-the-art improvements in the utility and design of models of respiratory disease, with a view to improving their translational potential and reducing wasteful animal usage is highlighted.
Journal ArticleDOI
Sustained activation of toll-like receptor 9 induces an invasive phenotype in lung fibroblasts: possible implications in idiopathic pulmonary fibrosis.
Varvara Kirillov,Jonathan T. Siler,Mahalakshmi Ramadass,Lingyin Ge,James N. Davis,Geraldine Grant,Steven D. Nathan,Gabor Jarai,Glenda Trujillo +8 more
TL;DR: Whether transforming growth factor (TGF)-β, a pleiotropic cytokine central to IPF pathogenesis, regulates TLR9 in lung myofibroblasts is investigated and results suggest a mechanism by which TGF-β andTLR9 responses in myofibiablasts collaborate to drive rapid progression of IPF.
Journal ArticleDOI
miR-323a-3p regulates lung fibrosis by targeting multiple profibrotic pathways
Lingyin Ge,David M. Habiel,Phil Hansbro,Richard Kim,Sina A. Gharib,Jeffery D. Edelman,Melanie Königshoff,Tanyalak Parimon,Rena Brauer,Ying Huang,Jenieke R. Allen,Dianhua Jiang,Adrianne Kurkciyan,Takako Mizuno,Barry R. Stripp,Paul W. Noble,Cory M. Hogaboam,Peter Chen +17 more
TL;DR: It is demonstrated that miR-323a-3p has a central role in lung fibrosis that spans across murine and human disease, and downregulated expression by the lung epithelium releases inhibition of various profibrotic pathways to promote fibroproliferation.
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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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