| Literature DB >> 29270465 |
Sara M Garrett1, DeAnna Baker Frost1, Carol Feghali-Bostwick1.
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.Entities:
Keywords: Extracellular matrix (ECM); Fibroblasts; Fibrosis; Scleroderma; Systemic sclerosis (SSc)
Year: 2017 PMID: 29270465 PMCID: PMC5736140 DOI: 10.5301/jsrd.5000240
Source DB: PubMed Journal: J Scleroderma Relat Disord ISSN: 2397-1983