Integrating mechanisms of pulmonary fibrosis
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TLDR
Pulmonary fibrosis is a complex and heterogeneous disease; a more detailed and integrated understanding of the cellular and molecular mechanisms influencing its pathogenesis will aid the design of new therapies.Abstract:
Pulmonary fibrosis is a highly heterogeneous and lethal pathological process with limited therapeutic options. Although research on the pathogenesis of pulmonary fibrosis has frequently focused on the mechanisms that regulate the proliferation, activation, and differentiation of collagen-secreting myofibroblasts, recent studies have identified new pathogenic mechanisms that are critically involved in the initiation and progression of fibrosis in a variety of settings. A more detailed and integrated understanding of the cellular and molecular mechanisms of pulmonary fibrosis could help pave the way for effective therapeutics for this devastating and complex disease.read more
Citations
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Journal ArticleDOI
Mechanisms of fibrosis: therapeutic translation for fibrotic disease
TL;DR: How cell-intrinsic changes in important structural cells can perpetuate the fibrotic response by regulating the differentiation, recruitment, proliferation and activation of extracellular matrix–producing myofibroblasts is described.
Journal ArticleDOI
The pathogenesis of cardiac fibrosis
TL;DR: Both experimental and clinical evidence suggests that cardiac fibrotic alterations may be reversible, and understanding the mechanisms responsible for initiation, progression, and resolution of cardiac fibrosis is crucial to design anti-fibrotic treatment strategies for patients with heart disease.
Journal ArticleDOI
Fibrosis — A Common Pathway to Organ Injury and Failure
TL;DR: The mechanisms underlying fibrosis and approaches to therapy are reviewed and fibrotic tissue becomes excessive, it can have diverse pathophysiological effects on a number of organ systems.
Journal ArticleDOI
Cellular senescence mediates fibrotic pulmonary disease
Marissa J. Schafer,Thomas A. White,Koji Iijima,Andrew J. Haak,Giovanni Ligresti,Elizabeth J. Atkinson,Ann L. Oberg,Jodie Birch,Hanna Salmonowicz,Yi Zhu,Daniel L. Mazula,Robert W. Brooks,Heike Fuhrmann-Stroissnigg,Tamar Pirtskhalava,Y. S. Prakash,Tamara Tchkonia,Paul D. Robbins,Marie Christine Aubry,João F. Passos,James L. Kirkland,Daniel J. Tschumperlin,Hirohito Kita,Nathan K. LeBrasseur +22 more
TL;DR: It is demonstrated that early-intervention suicide-gene-mediated senescent cell ablation improves pulmonary function and physical health, although lung fibrosis is visibly unaltered, and fibrotic lung disease is mediated, in part, by senescent cells.
Journal ArticleDOI
Single-Cell Transcriptomic Analysis of Human Lung Provides Insights into the Pathobiology of Pulmonary Fibrosis.
Paul A. Reyfman,James M. Walter,Nikita Joshi,Kishore R. Anekalla,Alexandra C. McQuattie-Pimentel,Stephen Chiu,Ramiro Fernandez,Mahzad Akbarpour,Ching I. Chen,Ziyou Ren,Rohan Verma,Hiam Abdala-Valencia,Kiwon Nam,Monica Chi,SeungHye Han,Francisco J. Gonzalez-Gonzalez,Saul Soberanes,Satoshi Watanabe,Kinola J.N. Williams,Annette S. Flozak,Trevor T. Nicholson,Vince K. Morgan,Deborah R. Winter,Monique Hinchcliff,Cara L. Hrusch,Robert D. Guzy,Catherine A. Bonham,Anne I. Sperling,Remzi Bag,Robert B. Hamanaka,Gökhan M. Mutlu,Anjana Yeldandi,Stacy A. Marshall,Ali Shilatifard,Luís A. Nunes Amaral,Harris Perlman,Jacob I. Sznajder,A. Christine Argento,Colin T. Gillespie,Jane Dematte,Manu Jain,Benjamin D. Singer,Karen M. Ridge,Anna P. Lam,Ankit Bharat,Sangeeta Bhorade,Cara J. Gottardi,G. R. Scott Budinger,Alexander V. Misharin +48 more
TL;DR: The results support the feasibility of discovery-based approaches using next-generation sequencing technologies to identify signaling pathways for targeting in the development of personalized therapies for patients with pulmonary fibrosis.
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