The significance of constitutive downregulation of Fli1 gene expression in the pathogenesis of dermal fibrosis and vasculopathy in systemic sclerosis

已有报道表明，系统性硬化症患者真皮成纤维细胞和真皮微血管内皮细胞的激活主要是由于Fl1基因的结构性下调，从而导致真皮纤维化和血管病变的发生。我们目前的研究表明，内皮素受体激动剂阻止SSC新的数字溃疡发展的可能靶点之一是转录因子FLI1，该转录因子的结构性下调与这种疾病的伤口愈合延迟有关。

Previous reports demonstrated that constitutive downregulation of Fli1 gene largely contributes to the activation of dermal fibroblasts and dermal microvascular endothelial cells in systemic sclerosis, leading to the development of dermal fibrosis and vasculopathy in this disease. Our current study revealed that one of the possible targets by which endothelin receptor actagonist prevents the development of new digital ulcers in SSc is transcription factor Fli1 and that constitutive downregulation of this transcription factor is associated with the delayed wound healing in this disease.