Tajima S, Yamada H
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Dermatology. 1995;191(2):115-8. doi: 10.1159/000246527.
The main clinical symptoms of systemic scleroderma are caused by an accumulation of collagen in the tissues. Familial scleroderma is a very rare disorder in which systemic scleroderma occurs in the members of the same family.
To know whether or not the biosynthetic properties of familial scleroderma skin fibroblasts are similar to those of nonfamilial scleroderma fibroblasts, syntheses of extracellular matrix components in the two fibroblast strains obtained from familial scleroderma patients were studied.
Expression of collagen and glycosaminoglycans by cultured familial scleroderma fibroblasts was determined.
Collagen synthesis by patient fibroblasts was elevated 1.4-fold compared with control fibroblasts. Translational activity of type I procollagen mRNA and the level of alpha 1 (I) mRNA in the patient fibroblasts was increased 1.7- and 1.6-fold, respectively. The ratio of hyaluronic acid/dermatan sulfate in the patient fibroblasts was lower than that in control fibroblasts.
The results indicate that the biosynthetic phenotypes of familial scleroderma fibroblasts are similar to those of nonfamilial scleroderma fibroblasts which have already been reported.
系统性硬皮病的主要临床症状是由组织中胶原蛋白的积累引起的。家族性硬皮病是一种非常罕见的疾病，其中系统性硬皮病发生在同一家族的成员中。
为了了解家族性硬皮病皮肤成纤维细胞的生物合成特性是否与非家族性硬皮病成纤维细胞相似，研究了从家族性硬皮病患者获得的两种成纤维细胞株中细胞外基质成分的合成。
测定培养的家族性硬皮病成纤维细胞中胶原蛋白和糖胺聚糖的表达。
与对照成纤维细胞相比，患者成纤维细胞的胶原蛋白合成增加了1.4倍。患者成纤维细胞中I型前胶原mRNA的翻译活性和α1（I）mRNA的水平分别增加了1.7倍和1.6倍。患者成纤维细胞中透明质酸/硫酸皮肤素的比例低于对照成纤维细胞。
结果表明，家族性硬皮病成纤维细胞的生物合成表型与已报道的非家族性硬皮病成纤维细胞相似。
