Koseki M, Ino S, Kimura A, Tsurumi K
Tohoku J Exp Med. 1981 Dec;135(4):431-9. doi: 10.1620/tjem.135.431.
Abnormal urinary excretion of sialoglycoconjugates was observed in four patients with mucopolysaccharidosis. Urinary sialoglycoconjugates were fractionated into 8 fractions by sequential gel filtration on Sephadex G-25, G-50 and by Dowex 1 ion-exchange chromatography. The comparison of the amounts of these fractions indicated that the fraction rich in mannose and glucosamine contents contributed to the increased urinary excretion of sialoglycoconjugates in patients with mucopolysaccharidosis. The major component of this fraction was disialyl-oroso-N-octaose which was a representative oligosaccharide side chain of glycoproteins with an Asn-GlcNAc linkage. Undoubtedly this abnormality is the secondary lesion of mucopolysaccharidosis, but it is conceivable that the disturbed metabolism of sialoglycoprotein is closely related to the pathogenesis of these diseases.
在4例黏多糖贮积症患者中观察到唾液酸糖共轭物的异常尿排泄。通过在Sephadex G - 25、G - 50上连续凝胶过滤以及Dowex 1离子交换色谱法，将尿唾液酸糖共轭物分离为8个组分。这些组分含量的比较表明，富含甘露糖和氨基葡萄糖的组分导致了黏多糖贮积症患者尿唾液酸糖共轭物排泄增加。该组分的主要成分是二唾液酸 - 奥索 - N - 八糖，它是具有Asn - GlcNAc连接的糖蛋白的代表性寡糖侧链。毫无疑问，这种异常是黏多糖贮积症的继发性病变，但可以想象，唾液酸糖蛋白代谢紊乱与这些疾病的发病机制密切相关。
