Fibroblasts are the most common cells of connective tissue. They produce the fibrous protein called collagen. "The major source of fibrosis in SSc is over production of collagens from fibroblasts."(1) ISN. (Also see What is Scleroderma? and Causes of Scleroderma)
Fibroblasts. Fibroblasts provide a structural framework (stroma) for many tissues, and play a critical role in wound healing. They are the most common cells of connective tissue in animals. Wikipedia.
Toll–like receptor 9 (TLR9) signaling is augmented in systemic sclerosis (SSc) and elicits transforming growth factor–ß (TGF–ß)–dependent fibroblast activation. In patients with SSc, mitochondrialDNA and other damage–associated TLR9 ligands in the skin might trigger localized activation of TLR9 signaling, TGF–ß production and consequent fibroblast activation. PubMed, Arthritis Rheumatol, 03/04/2016.
Multiplex cytokine analysis of dermal interstitial blister fluid defines local disease mechanisms in systemic sclerosis (SSc). An immuno-inflammatory environment and aberrant vascular repair are intimately linked to fibroblast activation in lesional skin in SSc. Arthritis Research and Therapy, 03/23/2015.
A reactive oxygen species(ROS)-mediated loop maintains the increased expression of NOX2 and NOX4 in skin fibroblasts from patients with systemic sclerosis (SSc). NOX2 and NOX4 generate ROS in SSc fibroblasts and play a critical role in cell activation and DNA damage. PubMed, Arthritis Rheumatol, 02/23/2015.
Role of Cellular Senescence and NOX4-Mediated Oxidative Stress in Systemic Sclerosis (SSc) Pathogenesis. Numerous studies have implicated oxidative stress in SSc pathogenesis, suggesting a plausible mechanism in which excessive oxidative stress induces cellular senescence and that the molecular events play an important role in the fibrotic and fibroproliferative vasculopathy characteristic of SSc. PubMed, Curr Rheumatol Rep, 2015 Jan;17(1):473.
Fibroblasts in fibrosis: novel roles and mediators. In addition to the extensive extracellular matrix fibroblasts have multiple concomitant biological roles, such as in wound healing, inflammation, and angiogenesis, which are each interwoven with the process of fibrosis. Pub Med Front Pharmacol, 2014 May 27;5:123. (Also see Wound Healing in Systemic Scleroderma)
Scleroderma (SSc) pathogenesis: a pivotal role for fibroblasts as effector cells. This article highlights the potentially important place of fibroblasts effector cells in fibrosis. Arthritis Research & Therapy 06/17/2013.
(1) Xiong M, Arnett FC, Guo X, Xiong H, Zhou X (2008) Differential Dynamic Properties of Scleroderma Fibroblasts in Response to Perturbation of Environmental Stimuli. PLoS ONE 3(2): e1693.
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