LeRoy E C
Department of Medicine, Medical University of South Carolina, Charleston, 29425.
Clin Exp Rheumatol. 1989 Sep-Oct;7 Suppl 3:S135-7.
Systemic sclerosis (scleroderma) is a generalized disease of the interstitial connective tissue of several organs characterized by vascular and microvascular injury and fibroblast activation leading to fibrosis. Cell culture models have been instructive in attempts to study the pathogenesis of this disorder. The scleroderma fibroblast in vitro demonstrates persistent, excessive extracellular matrix production, as well as an autocrine abnormality in cell growth regulation with persistent expression of the proto-oncogene c-myc. Since it has not yet been possible to culture scleroderma endothelial cells, human umbilical endothelial cells have been studied with regard to potential informational molecules (peptide regulatory factors, cytokines, growth factors) which selectively injure or alter the function of the endothelium. Transforming growth factor beta and tumor necrosis factor are two of these molecules which are currently under study.
系统性硬化症（硬皮病）是一种累及多个器官的间质性结缔组织的全身性疾病，其特征为血管和微血管损伤以及成纤维细胞活化导致纤维化。细胞培养模型对研究该疾病的发病机制具有指导意义。体外培养的硬皮病成纤维细胞表现出持续、过度的细胞外基质产生，以及细胞生长调节中的自分泌异常，原癌基因c-myc持续表达。由于目前尚无法培养硬皮病内皮细胞，因此对人脐内皮细胞进行了研究，以探寻可能选择性损伤或改变内皮功能的信息分子（肽调节因子、细胞因子、生长因子）。转化生长因子β和肿瘤坏死因子是目前正在研究的其中两种分子。
