Garrett Sara M, Baker Frost DeAnna, Feghali-Bostwick Carol
Department of Medicine, Division of Rheumatology and Immunology, Medical University of South Carolina, Charlestion, SC - USA.
J Scleroderma Relat Disord. 2017 May-Aug;2(2):69-134. doi: 10.5301/jsrd.5000240. Epub 2017 May 19.
Fibroblasts are the effector cells of fibrosis characteristic of systemic sclerosis (SSc, scleroderma) and other fibrosing conditions. The excess production of extracellular matrix (ECM) proteins is the hallmark of fibrosis in different organs, such as skin and lung. Experiments designed to assess the pro-fibrotic capacity of factors, their signaling pathways, and potential inhibitors of their effects that are conducted in fibroblasts have paved the way for planning clinical trials in SSc. As such, fibroblasts have proven to be valuable tools in the search for effective anti-fibrotic therapies for fibrosis. Herein we highlight the characteristics of fibroblasts, their role in the etiology of fibrosis, utility in experimental assays, and contribution to drug development and clinical trials in SSc.
成纤维细胞是系统性硬化症(SSc,硬皮病)和其他纤维化疾病所特有的纤维化效应细胞。细胞外基质(ECM)蛋白的过度产生是不同器官(如皮肤和肺)纤维化的标志。在成纤维细胞中进行的旨在评估各种因素的促纤维化能力、其信号通路以及其效应的潜在抑制剂的实验,为系统性硬化症的临床试验规划铺平了道路。因此,成纤维细胞已被证明是寻找有效的抗纤维化疗法治疗纤维化的宝贵工具。在此,我们重点介绍成纤维细胞的特征、它们在纤维化病因学中的作用、在实验分析中的用途以及对系统性硬化症药物开发和临床试验的贡献。