Arthritis Res Ther. 2010;12(3):125. doi: 10.1186/ar3026. Epub 2010 Jun 18.
Tissue fibrosis and vascular disease are hallmarks of systemic sclerosis (SSc). Transforming growth factor beta (TGFbeta) is a key-player in fibroblast activation and tissue fibrosis in SSc. In contrast to fibrosis, evidence for a role of TGFbeta in vascular disease of SSc is scarce. Using a transgenic mouse model with fibroblast-specific expression of a kinase-deficient TGFbeta receptor type II, Derrett-Smith and colleagues demonstrate that aberrant TGFbeta signaling in fibroblasts might result in activation of vascular smooth muscle cells and architectural changes of the vessel wall of the aorta.
组织纤维化和血管疾病是全身性硬皮病(SSc)的特征。转化生长因子β(TGFβ)是 SSc 中成纤维细胞激活和组织纤维化的关键因子。与纤维化相反,TGFβ 在 SSc 血管疾病中的作用证据很少。Derrett-Smith 及其同事使用成纤维细胞特异性表达激酶缺陷型 TGFβ 受体 II 的转基因小鼠模型表明,成纤维细胞中异常的 TGFβ 信号可能导致血管平滑肌细胞的激活和主动脉血管壁的结构改变。