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The mighty fibroblast and its utility in scleroderma research.

Authors :
Garrett SM
Baker Frost D
Feghali-Bostwick C
Source :
Journal of scleroderma and related disorders [J Scleroderma Relat Disord] 2017 May-Aug; Vol. 2 (2), pp. 69-134. Date of Electronic Publication: 2017 May 19.
Publication Year :
2017

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.<br />Competing Interests: Disclosures: Conflict of interest: None of the authors has financial interest related to this study.

Details

Language :
English
ISSN :
2397-1983
Volume :
2
Issue :
2
Database :
MEDLINE
Journal :
Journal of scleroderma and related disorders
Publication Type :
Academic Journal
Accession number :
29270465
Full Text :
https://doi.org/10.5301/jsrd.5000240