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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Journal ArticleDOI
Increased hemoglobin–oxygen affinity ameliorates bleomycin-induced hypoxemia and pulmonary fibrosis
Xin Geng,Kobina Dufu,Athiwat Hutchaleelaha,Qing Xu,Zhe Li,Chien-Ming Li,Mira Patel,Nicholas E. Vlahakis,Josh Lehrer-Graiwer,Donna Oksenberg +9 more
TL;DR: It is found that hemoglobin modification by GBT1118 had strong antihypoxemic therapeutic effects with improved arterial oxygen saturation to near normal level and significantly attenuated bleomycin‐induced lung fibrosis, collagen accumulation, body weight loss, and leukocyte infiltration.
Journal ArticleDOI
Loss of ELK1 has differential effects on age-dependent organ fibrosis
JT Cairns,Anthony Habgood,Rochelle C. Edwards-Pritchard,Chitra Joseph,Alison E. John,Chloe Wilkinson,Iain A. Stewart,Jack Leslie,Burns C. Blaxall,Katalin Susztak,Siegfried Alberti,Alfred Nordheim,Fiona Oakley,Gisli Jenkins,Amanda L. Tatler +14 more
TL;DR: A fundament role for ELK1 is supported in protecting against the development of progressive fibrosis via transcriptional regulation of beta integrin subunit genes, and it is demonstrated that loss of ELK 1 can be caused by cigarette smoke.
Journal ArticleDOI
An overview of the biology of a long-acting inhaled treprostinil prodrug
Richard W. Chapman,Michel R. Corboz,Vladimir Malinin,Adam J. Plaunt,Donna M. Konicek,Zhili Li,Walter Perkins +6 more
TL;DR: TPIS as discussed by the authors is a prostanoid analog pulmonary vasodilator drug that is formulated in a lipid nanoparticle (LNP) for administration as a nebulized inhaled suspension (TPIS).
Journal ArticleDOI
Organoid Model in Idiopathic Pulmonary Fibrosis.
TL;DR: The current organoids technology for human disease modeling, including lung organoids for IPF, is overviewed, with pluripotent stem cells-based organoid studies emerging as an alternative approach able to recapitulate tissue architecture with remarkable fidelity.
Journal ArticleDOI
Protein tyrosine phosphatase-α amplifies transforming growth factor-β-dependent profibrotic signaling in lung fibroblasts.
Yael Aschner,Yael Aschner,Meghan Nelson,Matthew Brenner,Helen Roybal,Keriann Beke,Carly Meador,Daniel Foster,Kelly A. Correll,Paul R. Reynolds,Kelsey Anderson,Elizabeth F. Redente,Elizabeth F. Redente,Jennifer Matsuda,David W. H. Riches,Steve D. Groshong,Ambra Pozzi,Ambra Pozzi,Jan Sap,Qin Wang,Dhaarmini Rajshankar,Christopher A. McCulloch,Rachel L. Zemans,Gregory P. Downey +23 more
TL;DR: It is suggested that PTPα amplifies pro-fibrotic TGF-β-dependent pathways signaling in lung fibroblasts and whether these pathways are relevant to human disease.
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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
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