Murata K, Takeda M
Clin Chim Acta. 1980 Nov 20;108(1):49-59. doi: 10.1016/0009-8981(80)90291-0.
The compositional changes of acidic glycosaminoglycans (AGAG) in the urine of progressive systemic sclerosis (PSS) patients were studied using chondroitinases and heparitinase in appropriate enzyme assays and by electrophoretic characterization. Daily urinary excretion of AGAG in patients with PSS was increased, when compared to normals. The proportion of urinary AGAG in PSS patients, which was undigested by chondroitinase-ABC (most probably representing heparan sulfates (HS)), increased significantly from the normal value. The substance was found to be mainly HS as determined by the electrophoretic pattern, thin-layer chromatographic analysis and by its susceptibility to heparitinase. After digestion of urinary chondroitin sulfate isomers with chondroitinases, the unsaturated disaccharides were mainly separated into 4-sulfated and 6-sulfated disaccharide units by paper chromatography. In all of the patients with PSS, the ratio of the unsaturated 4-sulfated disaccharide to the unsaturated disaccharide was higher than that in normal subjects. These observations indicate an abnormal turnover of AGAG in patients with PSS.
采用软骨素酶和肝素酶进行适当的酶分析，并通过电泳表征，研究了进行性系统性硬化症（PSS）患者尿液中酸性糖胺聚糖（AGAG）的组成变化。与正常人相比，PSS患者的AGAG每日尿排泄量增加。PSS患者尿液中未被软骨素酶ABC消化的AGAG比例（很可能代表硫酸乙酰肝素（HS））较正常值显著增加。通过电泳图谱、薄层色谱分析及其对肝素酶的敏感性确定，该物质主要为HS。用软骨素酶消化尿液中的硫酸软骨素异构体后，不饱和二糖通过纸色谱法主要分离为4-硫酸化和6-硫酸化二糖单元。在所有PSS患者中，不饱和4-硫酸化二糖与不饱和二糖的比例高于正常受试者。这些观察结果表明PSS患者AGAG的代谢异常。
