Kimura A, Hayashi S, Tsurumi K
Tohoku J Exp Med. 1980 Jul;131(3):227-39. doi: 10.1620/tjem.131.227.
Glycosaminoglycan isolated from the urine of a patient with the Hunter syndrome was composed of heparan sulfate (59.9%), dermatan sulfate (30.6%) and chondroitin sulfate (9.5%), and was heterogeneous in molecular weight (1,500-10,000) and in sulfate content (0.35-2.05 moles/mole of hexosamine). About 60% of dermatan sulfate and 10% of heparan sulfate had molecular weight of 7,000 to 10,000, while about 10% of the former and 60% of the latter had those of 1,500 to 3,500. Sulfate contents of dermatan sulfate and heparan sulfate were inversely related to their molecular weights. Higher total- and N-sulfate contents were measured in smaller molecular-weight heparan sulfate, and higher acetyl content was in larger molecular-weight heparan sulfate. On the basis of the chemical properties of dermatan sulfate and heparan sulfate isolated in this experiment, their catabolic processes in the Hunter syndrome were discussed.
从一名患有亨特综合征患者的尿液中分离出的糖胺聚糖，由硫酸乙酰肝素（59.9%）、硫酸皮肤素（30.6%）和硫酸软骨素（9.5%）组成，其分子量（1500 - 10000）和硫酸含量（0.35 - 2.05摩尔/摩尔己糖胺）存在异质性。约60%的硫酸皮肤素和10%的硫酸乙酰肝素分子量为7000至10000，而前者约10%和后者约60%的分子量为1500至3500。硫酸皮肤素和硫酸乙酰肝素的硫酸含量与它们的分子量呈负相关。在分子量较小的硫酸乙酰肝素中测得较高的总硫酸和N - 硫酸含量，而在分子量较大的硫酸乙酰肝素中乙酰含量较高。基于本实验中分离出的硫酸皮肤素和硫酸乙酰肝素的化学性质，讨论了它们在亨特综合征中的分解代谢过程。
