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

以往的研究表明，Fli1基因的组成性下调在系统性硬化症中很大程度上促进了真皮成纤维细胞和真皮微血管内皮细胞的活化，导致了该疾病中真皮纤维化和血管病变的发展。我们目前的研究表明，内皮素受体激动剂预防SSc中新的指溃疡发展的可能靶点之一是转录因子Fli1，并且该转录因子的组成性下调与这种疾病中伤口愈合延迟相关。

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.