Uysal H, Hemming F W
School of Biomedical Sciences, University of Nottingham, Queen's Medical Centre, Nottingham NG7 2UH, U.K.
Br J Dermatol. 1999 Oct;141(4):658-66. doi: 10.1046/j.1365-2133.1999.03104.x.
Fibronectin, laminin and tenascin play an important part in the assembly of the extracellular matrix and the interaction of cells with it. In this study, changes in their expression and distribution associated with tuberous sclerosis are reported. Fibroblasts from three different tuberous sclerosis skin lesions (forehead plaque, neck fibroma and ungual fibroma) secreted more fibronectin and tenascin into their culture medium than did normal skin fibroblasts. Immunohistochemistry and flow cytometry showed that cells from an ungual fibroma which secreted most of each of these glycoproteins also retained more of them, associated mainly with the cell surface and a perinuclear area. Laminin was also produced by all fibroblasts but only in those from the neck fibroma was more both secreted and retained. The proportions of fibronectin/laminin/tenascin secreted by the skin lesion fibroblasts were markedly different from normal. The results suggest that the characteristic tissue hardening of skin lesions in tuberous sclerosis may result, at least in part, from differences in the expression and distribution of these critical components of the extracellular matrix and its consequent abnormal assembly.
纤连蛋白、层粘连蛋白和腱生蛋白在细胞外基质的组装以及细胞与之相互作用中发挥着重要作用。在本研究中，报告了它们与结节性硬化症相关的表达和分布变化。来自三种不同结节性硬化症皮肤病变（额头斑块、颈部纤维瘤和甲周纤维瘤）的成纤维细胞向其培养基中分泌的纤连蛋白和腱生蛋白比正常皮肤成纤维细胞更多。免疫组织化学和流式细胞术显示，来自甲周纤维瘤的细胞分泌了这些糖蛋白中的大部分，并且还保留了更多，主要与细胞表面和核周区域相关。所有成纤维细胞也都产生层粘连蛋白，但只有颈部纤维瘤的成纤维细胞分泌和保留的层粘连蛋白更多。皮肤病变成纤维细胞分泌的纤连蛋白/层粘连蛋白/腱生蛋白比例与正常情况明显不同。结果表明，结节性硬化症皮肤病变的特征性组织硬化可能至少部分是由于细胞外基质这些关键成分的表达和分布差异及其随后的异常组装所致。
