β-转化生长因子及其受体在硬皮病中的意义

Transforming growth factor-β and its receptors in scleroderma

系统性硬皮病(SSc)病因不清,以成纤维细胞过度合成细胞外基质(ECM)并沉积于皮肤及肺部,导致组织纤维化为主要特征。目前认为,β-转化生长因子(TGFβ)及其受体在硬皮病发病机制中起重要作用。TGFβ通过与成纤维细胞上的受体结合,激活Smads信号通路,上调细胞外基质的基因转录和翻译,诱导SSc病变;并在多种辅助受体及细胞因子的影响下,最终导致疾病发生。探索针对SSc中TGFβ通路的特异性治疗方法,在纤维化疾病临床治疗中具有重要意义。

The hallmark of scleroderma is fibrosis by excessive extracellular matrix （ECM） deposition in the skin,lung,and other organs. Increasing evidence suggests that overexpression of transforming growth factor-β （TGF-β） and its receptors play a key pathogenic role in the development of tissue fibrosis in scleroderma. TGF-β is known to induce the expression of ECM proteins in the pathogenesis of fibrosis in systemic sclerosis. Investigations into TGF-βpathways will suggest new treatment strategies for fibrotic diseases.