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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 etiology of systemic scleroderma (SSc) is unclear, with fibroblasts over-synthesizing extracellular matrix (ECM) and depositing in the skin and lungs, leading to tissue fibrosis as the main feature. At present, it is thought that β-transforming growth factor (TGFβ) and its receptor plays an important role in the pathogenesis of scleroderma. TGFβ binds to fibroblasts, activates Smads signaling, upregulates extracellular matrix gene transcription and translation, and induces SSc pathology. TGFβ eventually leads to disease under the influence of various co-receptors and cytokines. To explore the specific treatment of TGFβ pathway in SSc is of great significance in the clinical treatment of fibrotic diseases.