Duksin D, Gal A, Goodman R M
Lab Invest. 1983 Sep;49(3):346-52.
Skin fibroblasts, from a 9-year-old girl with an apparent heritable disorder of connective tissue, were cultured in vitro. The biosynthesis of the extracellular matrix glycoproteins, procollagens, and fibronectin was studied using radioactively labeled sugars and amino acids. Glycoproteins synthesized and secreted into the growth medium were found to be only partially glycosylated and the extracellular limited proteolytic conversion of procollagen type I to collagen was impaired, with procollagen chains accumulating in the growth medium. These biosynthetic alterations have not been previously reported in human skin fibroblasts, but similar findings have been described in tunicamycin-treated chick fibroblasts.
从一名患有明显遗传性结缔组织疾病的9岁女孩身上获取皮肤成纤维细胞，并在体外进行培养。利用放射性标记的糖类和氨基酸研究了细胞外基质糖蛋白、前胶原和纤连蛋白的生物合成。发现合成并分泌到生长培养基中的糖蛋白仅部分糖基化，而且I型前胶原向胶原的细胞外有限蛋白水解转化受损，前胶原链在生长培养基中积累。这些生物合成改变此前在人皮肤成纤维细胞中尚未见报道，但在衣霉素处理的鸡成纤维细胞中曾有过类似发现。
