Koseki M, Wu J Y, Tsurumi K, Nagai Y
Tohoku J Exp Med. 1978 Apr;124(4):351-60. doi: 10.1620/tjem.124.351.
Low molecular sialoglucides were isolated from the urines of normal human male and two patients with lysosomal disease (mucopolysaccharidosis type II and a new type of mucolipidosis) by charcoal adsorption method. Urinary sialoglucides were fractionated into two fractions (SG-1 and SG-2) by Sephadex G-25 gel filtration and considerable increase in excretion of SG-1 was observed in the patients with lysosomal diseases: two- to three-fold increase in mucopolysaccharidosis type II and seven- to eight-fold increase in mucolipidosis. SG-1 was further fractionated into 18 to 19 fractions by Sephadex G-50 gel filtration and ion exchange chromatography. Comparison of the amounts and the chemical compositions of these fractions suggested that the increase in SG-1 was dependent upon the increase in excretion of low molecular sialoglucides rich in mannose and N-acetylglucosamine.
采用活性炭吸附法从正常男性尿液以及两名溶酶体病患者（II型粘多糖贮积症和一种新型粘脂贮积症）的尿液中分离出低分子唾液酸糖。通过Sephadex G - 25凝胶过滤将尿唾液酸糖分为两个部分（SG - 1和SG - 2），并且在溶酶体病患者中观察到SG - 1排泄量显著增加：II型粘多糖贮积症患者增加了两到三倍，粘脂贮积症患者增加了七到八倍。通过Sephadex G - 50凝胶过滤和离子交换色谱法将SG - 1进一步分为18至19个部分。这些部分的量和化学组成的比较表明，SG - 1的增加取决于富含甘露糖和N - 乙酰葡糖胺的低分子唾液酸糖排泄量的增加。
