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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Esomeprazole Alleviates Fibrosis in Systemic Sclerosis by Modulating AhR/Smad2/3 Signaling
Jiani Liu,Zixin Pi,Yangfan Xiao,Zhuotong Zeng,Jiangfan Yu,Puyu Zou,Bingsi Tang,Xiangning Qiu,Rui Tang,Yaqian Shi,Rong Xiao +10 more
TL;DR: In this paper , the effects of esomeprazole on SSc and its underlying mechanisms were investigated, and it was shown that ESO suppresses the migration of SSc dermal fibroblasts, downregulates profibrotic markers, including COLIA1, α-SMA CTGF and MMP1.
Journal ArticleDOI
Elastase modifies bleomycin-induced pulmonary fibrosis in mice.
Larissa Alexsandra da Silva Neto Trajano,Eduardo Tavares Lima Trajano,Manuella Lanzetti,Morena Scopel Amorim Mendonça,Rafael F. Guilherme,Rodrigo T. Figueiredo,Claudia F. Benjamim,Samuel Santos Valença,Andréa Monte Alto Costa,Luís Cristóvão Porto +9 more
TL;DR: PF and emphysema did not coexist in the authors' model of lung disease and despite increased levels of oxidative stress and inflammatory markers after combined stimulus (elastase and bleomycin) overall histology was improved to that of the nearest control group.
Journal ArticleDOI
Cell tracing reveals the transdifferentiation fate of mouse lung epithelial cells during pulmonary fibrosis in vivo.
TL;DR: In this paper, the expression of Cre recombinase and SPC was analyzed using immunohistochemical (IHC) staining to detect the labeling efficiency in the peripheral lung tissue was 63.27±7.51%.
Journal ArticleDOI
Accelerated aging induced by deficiency of Zmpste24 protects old mice to develop bleomycin-induced pulmonary fibrosis.
Jazmin Calyeca,Yalbi I. Balderas-Martínez,Raúl Olmos,Rogelio Jasso,Vilma Maldonado,Quetzali Rivera,Moisés Selman,Annie Pardo +7 more
TL;DR: The results indicate that the absence of Zmpste24 in aging mice results in impaired lung fibrotic response after injury, which is likely associated to the dysregulation of fibrosis-related miRNAs.
Journal ArticleDOI
Insights gained in the pathology of lung disease through single‐cell transcriptomics
Gianni Carraro,Barry R. Stripp +1 more
TL;DR: Recent advances in the understanding of disease‐related changes in the molecular phenotype of human lung epithelium that have emerged from single‐cell transcriptomic studies are summarized and emerging concepts of epithelial transitional states that characterize the pathological remodeling that accompanies chronic lung diseases are focused on.
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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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