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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.

SUBMITTER: Garrett SM

PROVIDER: S-EPMC5736140 | biostudies-literature | 2017 May-Aug

REPOSITORIES: biostudies-literature

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The mighty fibroblast and its utility in scleroderma research.

Garrett Sara M SM Baker Frost DeAnna D Feghali-Bostwick Carol C

Journal of scleroderma and related disorders 20170519 2

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 su ...[more]

PMID: 29270465

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