REGULATION OF EARLY INFLAMMATION BY MINDIN IS ESSENTIAL FOR CUTANEOUS FIBROGENESIS IN A NEW MOUSE MODEL OF SYSTEMIC SCLEROSIS

Fibrosis is a result of chronically activated fibroblasts leading to the overproduction of extracellular matrix (ECM), causing tissue hardening and loss of organ function. A fibrotic disease affecting the skin is Systemic sclerosis (SSc) and is marked by inflammation, autoimmunity and vasculopathy along with progressive fibrosis of the skin and internal organs. A major bottleneck in understanding the etiology of SSc has been the lack of a holistic animal model that can mimic the human SSc disease. We found that the transcription factor Snail is overexpressed in the epidermis of SSc patients and a transgenic mouse recapitulating thisexpression pattern is sufficient to induce hallmark clinical features of the human disease.