Steijlen P M, Maessen E, Kresse H, Van Vlijmen I M, Verstraeten A A, Traupe H, Schalkwijk J
Department of Dermatology, University Hospital Nijmegen, The Netherlands.
Br J Dermatol. 1994 May;130(5):564-8. doi: 10.1111/j.1365-2133.1994.tb13100.x.
The distribution of three (recently discovered) extracellular matrix components (tenascin, biglycan and decorin) was studied in normal adult human skin and in a number of monogenic disorders of keratinization, using immunohistology. The expression of tenascin, which is sparsely distributed in normal human dermis, was found to be grossly increased in epidermolytic hyperkeratoses and in Darier's disease. Tenascin expression in three types of ichthyosis (X-linked recessive ichthyosis, autosomal dominant ichthyosis vulgaris, non-erythrodermic lamellar ichthyosis) was similar to that of normal skin. The presence of biglycan and decorin did not show a marked variation between the different disorders studied, suggesting that their expression is subject to regulatory mechanisms distinct from those of tenascin. The increased expression of tenascin in two disorders of keratinization with a hyperproliferative phenotype, lends further support to the hypothesis that dermal tenascin expression is increased as a result of epidermal hyperproliferation.
利用免疫组织学方法，研究了三种（最近发现的）细胞外基质成分（腱生蛋白、双糖链蛋白聚糖和核心蛋白聚糖）在正常成人皮肤以及一些单基因角化异常疾病中的分布情况。腱生蛋白在正常人真皮中分布稀疏，发现在表皮松解性角化过度症和达里埃病中其表达显著增加。在三种鱼鳞病（X连锁隐性鱼鳞病、常染色体显性寻常型鱼鳞病、非红皮病性板层状鱼鳞病）中腱生蛋白的表达与正常皮肤相似。双糖链蛋白聚糖和核心蛋白聚糖的存在在所研究的不同疾病之间未显示出明显差异，这表明它们的表达受不同于腱生蛋白的调节机制调控。在两种具有增殖过度表型的角化异常疾病中腱生蛋白表达增加，进一步支持了以下假说：由于表皮增殖过度，真皮腱生蛋白表达增加。
