Vuorio T, Kähäri V M, Black C, Vuorio E
Department of Medical Biochemistry, University of Turku, Finland.
J Rheumatol. 1991 Feb;18(2):247-51.
A characteristic feature of fibroblasts cultured from affected skin areas of patients with systemic sclerosis (SSc) and localized scleroderma (morphea) is excessive activation of collagen biosynthesis. To elucidate the nature of fibroblast activation in scleroderma we have studied the expression of 3 noncollagenous connective tissue components, osteonectin, small dermatan sulfate proteoglycan (proteoglycan II, decorin), and transforming growth factor-beta 1 (TGF-beta 1), by measuring their mRNA levels in fibroblast cultures from 6 patients with SSc and 3 with morphea. A clear correlation was observed between the increase in type I collagen and osteonectin mRNA in these cell lines. The apparent overproduction of osteonectin by scleroderma fibroblasts is in accordance with the suggested activation of osteonectin expression during tissue remodeling. The levels of decorin mRNA showed marked variation in the cell lines, but were in no correlation with collagen or osteonectin mRNA. The levels of TGF-beta 1 mRNA were found to be slightly elevated in fibroblasts grown from affected scleroderma skin. This may suggest that this potent activator of collagen production has a role during the initial activation of dermal fibroblasts both in SSc and morphea.
从系统性硬化症(SSc)和局限性硬皮病(硬斑病)患者受影响皮肤区域培养的成纤维细胞的一个特征是胶原生物合成过度激活。为了阐明硬皮病中成纤维细胞激活的本质,我们通过测量6例SSc患者和3例硬斑病患者的成纤维细胞培养物中3种非胶原性结缔组织成分骨连接蛋白、小硫酸皮肤素蛋白聚糖(蛋白聚糖II,核心蛋白聚糖)和转化生长因子-β1(TGF-β1)的mRNA水平,对其进行了研究。在这些细胞系中,观察到I型胶原和骨连接蛋白mRNA的增加之间存在明显的相关性。硬皮病成纤维细胞中骨连接蛋白的明显过量产生与组织重塑过程中骨连接蛋白表达的激活相一致。核心蛋白聚糖mRNA的水平在细胞系中显示出明显的变化,但与胶原或骨连接蛋白mRNA无关。发现从硬皮病受影响皮肤生长的成纤维细胞中TGF-β1 mRNA水平略有升高。这可能表明这种强大的胶原产生激活剂在SSc和硬斑病的真皮成纤维细胞初始激活过程中起作用。