Barlati S, Moro L, Gardella R, Colombi M
Department of Biomedical Sciences and Biotechnologies, University of Brescia, Italy.
Cell Biol Int Rep. 1991 Dec;15(12):1183-94. doi: 10.1016/0309-1651(91)90090-6.
In vitro cultured skin fibroblasts derived from Ehlers-Danlos Syndrome (EDS) type I to VIII patients lack fibronectin-containing extracellular matrix (FN-ECM) which can be restored when EDS cells are cocultivated over a feeder of control fibroblasts. Further analysis, focused on EDS types III and IV cells, showed that partial matrix correction in EDS type III cells can be obtained by their cultivation over a feeder of EDS type IV fibroblasts, but not vice versa. An apparently normal FN-ECM can be restored in EDS types III and IV cells also by the addition of cellular--but not plasma--FN. These biological features might be used for a better understanding of ECM assembly and for the characterization of the different EDS cell types.
从I型至VIII型埃勒斯-当洛综合征（EDS）患者身上获取的体外培养皮肤成纤维细胞缺乏含纤连蛋白的细胞外基质（FN-ECM），而当EDS细胞与对照成纤维细胞饲养层共培养时，这种基质能够得以恢复。进一步针对III型和IV型EDS细胞的分析表明，III型EDS细胞通过在IV型EDS成纤维细胞饲养层上培养可实现部分基质校正，但反之则不行。通过添加细胞型纤连蛋白而非血浆型纤连蛋白，也能够在III型和IV型EDS细胞中恢复明显正常的FN-ECM。这些生物学特性或许可用于更好地理解细胞外基质组装以及对不同类型的EDS细胞进行表征。
