Cutolo M, Castellani P, Borsi L, Zardi L
Clin Exp Rheumatol. 1986 Apr-Jun;4(2):125-8.
Using the immunofluorescence technique we have studied the distribution of fibronectin in cultured fibroblasts from patients affected by Ehlers-Danlos Syndrome (EDS) types I, II and VI. In these cells the amount of fibronectin production is reduced with respect to normal fibroblasts; moreover fibronectin fibers are shorter, thicker, mainly pericellular and show intracytoplasmic accumulation. The altered fibronectin distribution may be the result of altered interactions of fibronectin with extracellular matrix components, either due to abnormal fibronectin or to changes in other extracellular matrix molecules (e.g. collagens, hyaluronic acid).
我们运用免疫荧光技术研究了患有Ⅰ型、Ⅱ型和Ⅵ型埃勒斯-当洛综合征（EDS）患者培养的成纤维细胞中纤连蛋白的分布情况。与正常成纤维细胞相比，这些细胞中纤连蛋白的产生量减少；此外，纤连蛋白纤维更短、更粗，主要分布在细胞周围，并表现出胞质内积累。纤连蛋白分布的改变可能是由于纤连蛋白与细胞外基质成分的相互作用改变所致，这可能是由于纤连蛋白异常或其他细胞外基质分子（如胶原蛋白、透明质酸）发生变化引起的。
