Hultberg B
Hum Hered. 1979;29(4):250-6. doi: 10.1159/000153053.
The incorporation of 14C-D-mannose or 14C-L-fucose into cultured fibroblasts from controls and patients with mannosidosis and mucolipidosis type II was studied. Mannose-containing oligosaccharide chains retarded on Sephadex G-25 were accumulated in cells from both patients. Fucose-containing oligosaccharides retarded on G-25 were also accumulated in cells from the patient with mucolipidosis. Besides there was an increase of macromolecular fucose-containing material in the cells from the patient with mucolipidosis. Conditioned medium of the cells from controls and the different patients exhibited about the same pattern as found in the cells, which might indicate leakage of the cellular material due to cell damage or death.
研究了将¹⁴C-D-甘露糖或¹⁴C-L-岩藻糖掺入来自对照、甘露糖苷贮积症和II型粘脂贮积症患者的培养成纤维细胞中的情况。在Sephadex G-25上滞留的含甘露糖寡糖链在两名患者的细胞中均有积累。在G-25上滞留的含岩藻糖寡糖也在粘脂贮积症患者的细胞中积累。此外，粘脂贮积症患者细胞中含岩藻糖的大分子物质有所增加。对照和不同患者细胞的条件培养基表现出与细胞中发现的大致相同的模式，这可能表明由于细胞损伤或死亡导致细胞物质泄漏。
