Takahashi Y, Orii T
Department of Pediatrics, Gifu University School of Medicine, Japan.
Clin Chim Acta. 1989 Feb 15;179(2):153-62. doi: 10.1016/0009-8981(89)90161-7.
Urinary oligosaccharides of GM1 gangliosidosis patients (type 2A, 4 cases; type 2B, 2 cases) were investigated using the Bio Gel system. The levels of urinary oligosaccharide excreted (nmol/mg creatinine) by the type 2A patients were 4.1 times the levels of the type 2B patients. Patients of type 2A excreted high molecular weight oligosaccharides which were not detected in the urine of type 2B, and excreted oligosaccharides with long linkages of repeating structures. Thus, type 2A apparently has biochemically different characteristics from type 2B related to urinary oligosaccharide. Differentiation of type 2A from type 2B can thus be made biochemically. The structures of 5 different kinds of oligosaccharides not reported previously were confirmed.
使用生物凝胶系统对GM1神经节苷脂贮积症患者（2A型，4例；2B型，2例）的尿寡糖进行了研究。2A型患者排泄的尿寡糖水平（纳摩尔/毫克肌酐）是2B型患者的4.1倍。2A型患者排泄出2B型患者尿液中未检测到的高分子量寡糖，并且排泄出具有重复结构长链连接的寡糖。因此，2A型在尿寡糖方面显然具有与2B型不同的生化特征。由此可以通过生化方法区分2A型和2B型。确认了5种先前未报道的不同寡糖的结构。
