Shinkai H, Hirabayashi O, Tamaki A, Matsubayashi S, Sano S
Arch Dermatol Res (1975). 1976 Dec 15;257(2):113-23. doi: 10.1007/BF00558084.
Study on connective tissue metabolism was conducted with a patient with Ehlers-Danlos syndrome (E-D) of Type I who visited our institute. The conversion of procollagen into tropocollagen in the medium of cultured fibroblasts was assayed by the chase technique using 3H-proline. The conversion was inhibited in the cultured fibroblasts of the patient. The components of glycosaminoglycans in cultured medium and fibroblasts from E-D were within normal ranges, however, the ratio of glycoprotein to glycosaminoglycans of intra- and extra-cellular fractions of E-D fibroblasts was higher than the normal one. These findings suggest that the insufficient maturation of collagen fiber may be considered fundamental disorders of E-D.
对一名前来我院就诊的Ⅰ型埃勒斯-当洛综合征（E-D）患者进行了结缔组织代谢研究。采用3H-脯氨酸追踪技术检测培养的成纤维细胞培养基中前胶原向原胶原的转化。该患者培养的成纤维细胞中的转化受到抑制。E-D患者培养基和成纤维细胞中糖胺聚糖的成分在正常范围内，然而，E-D成纤维细胞细胞内和细胞外部分糖蛋白与糖胺聚糖的比例高于正常水平。这些发现表明，胶原纤维成熟不足可能被认为是E-D的基本病症。
