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应用电泳、酶或化学处理及离子交换柱层析分析了我国两例(胞兄妹)Sanfilippo综合征尿氨基多糖的生化特点。两患者的尿氨基多糖主要为硫酸乙酰肝素,分别占71%及58%,而硫酸软骨素则占29%及42%。患者尿硫酸乙酰肝素经纯化后测其C/O比值为2.32,显示其在己糖醛酸组成上与一般硫酸乙酰肝素无明显差异.但DEAE-SephadexA-25柱层析及在0.1M HCl中的电泳分析,均说明患者排泄的硫酸乙酰肝素是不均一的,它至少有两种硫酸基及电荷密度不同的分子类型。
The biochemical characteristics of aminopolysaccharide in urine of Sanfilippo syndrome in two cases in China were analyzed by electrophoresis, enzyme or chemical treatment and ion exchange column chromatography. Urinary amylase was mainly heparan sulfate in both patients, accounting for 71% and 58%, respectively, while chondroitin sulfate accounted for 29% and 42%, respectively. The urinary heparan sulfate was purified to give a C / O ratio of 2.32 and showed no significant difference in the hexuronic acid composition from the usual heparan sulfate but in DEAE-Sephadex A-25 column chromatography and in 0.1 M HCl Electrophoresis analysis, all show patients excreted heparan sulfate is heterogeneous, it has at least two kinds of sulfate groups and different charge-density molecular types.