Kimura A, Hayashi S, Tsurumi K
Tohoku J Exp Med. 1980 Jul;131(3):241-7. doi: 10.1620/tjem.131.241.
The chemical structure of dermatan sulfate (DS) in the urine of a patient the Hunter syndrome was studied through the analysis of disaccharide units which were derived from the urinary DS by digestion with chondroitinase ABC and separated on a Dowex 1 column. The DS was basically composed of repeating disaccharide units of iduronyl N-acetylgalactosamine 4-sulfate. About 90% of the excess sulfate were linked to the iduronate residues as an additional sulfate group in the unit. N-Acetylgalactosamine 6-sulfate and N-acetylgalactosamine 4,6-disulfate residues were minor components. No non-sulfated disaccharide unit was detected in the digestion products. Only sulfoiduronate residue was found as the non-reducing terminal sugar of the DS molecule, consistent with the lack of iduronosulfate sulfatase in this disease.
通过对二糖单元的分析，研究了一名患有亨特综合征患者尿液中硫酸皮肤素（DS）的化学结构。这些二糖单元是通过用软骨素酶ABC消化尿液中的DS得到的，并在Dowex 1柱上进行分离。DS基本上由艾杜糖醛酸N-乙酰半乳糖胺4-硫酸酯的重复二糖单元组成。约90%的过量硫酸盐作为单元中的一个额外硫酸基团与艾杜糖醛酸残基相连。N-乙酰半乳糖胺6-硫酸酯和N-乙酰半乳糖胺4,6-二硫酸酯残基是次要成分。在消化产物中未检测到非硫酸化的二糖单元。仅发现硫酸艾杜糖醛酸残基作为DS分子的非还原末端糖，这与该疾病中缺乏艾杜糖醛酸硫酸酯酶一致。
