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The mighty fibroblast and its utility in scleroderma research

Abstract

Fibroblasts are the effector cells of fibrosis characteristic of systemic sclerosis (SSc, scleroderma) and other fibrosing conditions. The excess production of extracellular matrix (ECM) proteins is the hallmark of fibrosis in different organs, such as skin and lung. Experiments designed to assess the pro-fibrotic capacity of factors, their signaling pathways, and potential inhibitors of their effects that are conducted in fibroblasts have paved the way for planning clinical trials in SSc. As such, fibroblasts have proven to be valuable tools in the search for effective anti-fibrotic therapies for fibrosis. Herein we highlight the characteristics of fibroblasts, their role in the etiology of fibrosis, utility in experimental assays, and contribution to drug development and clinical trials in SSc.

J scleroderma relat disord 2017; 2(2): 100 - 107

Article Type: REVIEW

DOI:10.5301/jsrd.5000240

Authors

Sara M. Garrett, DeAnna Baker Frost, Carol Feghali-Bostwick

Article History

Disclosures

Financial support: This work is funded in part by NIH grants K24AR060297 and R01 HL121262.
Conflict of interest: None of the authors has financial interest related to this study.

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Authors

Affiliations

  • Department of Medicine, Division of Rheumatology & Immunology, Medical University of South Carolina, Charleston, SC - USA

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