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Avouac, Jérôme
Methods in molecular biology (Clifton, N.J.), 01/2014, Volume: 1142Journal Article
Relevant animal models are essential tools to investigate in depth the pathogenesis of autoimmune disease. Systemic sclerosis (SSc) is an autoimmune connective tissue disorder that affects particularly the skin. SSc is characterized by vasculopathy, immune disturbances, and fibrosis. Expression of each of the three pathologic features varies among SSc patients leading to disease heterogeneity and variable organ manifestations. Several animal models of SSc are available; however, some models display inflammation followed by fibrosis, whether some others primarily mimic autonomous fibroblast activation. Here, we describe the mouse model of bleomycin-induced dermal fibrosis, which mimics early and inflammatory stages of SSc, and is widely used in SSc research.
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