URINARY ACID-MUCOPOLYSACCHARIDES IN MULTIPLE SULFATASE DEFICIENCY (MUCOSULFATIDOSIS)

被引：9

作者：

ETO, Y ^{[1
]}

NUMAGUCHI, S ^{[1
]}

HANDA, T ^{[1
]}

机构：

[1] HIGASHISAITAMA NATL SANAT HOSP,DEPT PEDIAT,SAITAMA,JAPAN

来源：

EUROPEAN JOURNAL OF PEDIATRICS | 1979年 / 132卷 / 03期

关键词：

Chondroitin sulfate A/C; Heparan sulfate; Metachromatic leukodystrophy; Multiple sulfatase deficiencies; Urinary acid mucopolysaccharide;

D O I：

10.1007/BF00442437

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

Urinary acid mucopolysaccharide (AMPS) excretion was investigated in a Japanese case with Multiple Sulfatase Deficiency (MSD) (Mucosulfatidosis). The patient excreted AMPS 4 to 5 times more (as carbazoluronic acid) than controls. The cellulose acetate gel electrophoresis clearly indicated two major AMPS which co-migrated with heparan sulfate and chondroitin sulfate A/C. Enzymic digestion with chondroitinase AC and ABC, and by testicular hyaluronidase plus amino sugar analysis also confirmed that our case excreted heparan sulfate and chondroitin sulfate A/C. These findings suggest that there are heterogeneities of urinary AMPS excretion among cases with MSD. © 1979 Springer-Verlag.

引用

页码：207 / 211

页数：5

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