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
Generation of an alveolar epithelial type II cell line from induced pluripotent stem cells.
Luca Tamò,Youssef Hibaoui,Sampada Kallol,Marco P. Alves,Christiane Albrecht,Katrin Hostettler,Anis Feki,Jean-Sébastien Rougier,Hugues Abriel,Lars Knudsen,Amiq Gazdhar,Thomas Geiser +11 more
TL;DR: A long-lasting, but finite AEC type II cell line derived from iPSC is established as a novel cellular model to study alveolar epithelial cell biology in lung health and disease.
Journal ArticleDOI
Role of fibroblast growth factor 23 and klotho cross talk in idiopathic pulmonary fibrosis.
Jarrod W. Barnes,Dawn Duncan,Scott Helton,Samuel B. Hutcheson,Deepali Kurundkar,Naomi J. Logsdon,Morgan L. Locy,Jaleesa M. Garth,R. Denson,Carol Farver,Hai T. Vo,Gwendalyn D. King,Dominik Kentrup,Christian Faul,Tejaswini Kulkarni,Joao A. de Andrade,Joao A. de Andrade,Zhihong Yu,Sadis Matalon,Victor J. Thannickal,Stefanie Krick +20 more
TL;DR: In IPF, downregulation of KL may contribute to fibrosis and inflammation and FGF23 may act as a compensatory anti-fibrotic and anti-inflammatory mediator via inhibition of TGF-β signaling, which opens new avenues for potential therapies in aging-related diseases like IPF.
Journal ArticleDOI
Targeted inhibition of PI3 kinase/mTOR specifically in fibrotic lung fibroblasts suppresses pulmonary fibrosis in experimental models
Suraj U. Hettiarachchi,Yen Hsing Li,Jyoti Roy,Fenghua Zhang,Estela Puchulu-Campanella,Spencer D. Lindeman,Madduri Srinivasarao,Konstantin Tsoyi,Konstantin Tsoyi,Xiaoliang Liang,Xiaoliang Liang,Ehab A. Ayaub,Cheryl Nickerson-Nutter,Ivan O. Rosas,Ivan O. Rosas,Philip S. Low +15 more
TL;DR: It is demonstrated here that a low–molecular weight FAP ligand can be used to deliver imaging and therapeutic agents selectively to FAP-expressing cells and suggest that a F AP-targeted PI3K inhibitor might be promising for treating IPF.
Journal ArticleDOI
Pathogenesis of idiopathic pulmonary fibrosis and its clinical implications
TL;DR: It is shown that smoking, exposure to organic and inorganic dust and genetic factors have been shown to increase the risk of disease, although the cause of IPF remains elusive and its pathogenesis incompletely understood.
Journal ArticleDOI
Radiation induced pulmonary fibrosis as a model of progressive fibrosis: Contributions of DNA damage, inflammatory response and cellular senescence genes.
TL;DR: The validity of RIPF as a model of progressive PF/IPF based on the numbers of transcripts reported in both literature and observed experimentally is illustrated, supporting the hypotheses that DNA damage, inflammatory response and cellular senescence are associated with the pathogenesis of PF.
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Journal ArticleDOI
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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