GENERALIZED GANGLIOSIDOSIS - IMPAIRED CLEAVAGE OF GALACTOSE FROM A MUCOPOLYSACCHARIDE AND A GLYCOPROTEIN

被引：49

作者：

MACBRINN, MC

OKADA, S

HO, MW

HU, CC

OBRIEN, JS

机构：

来源：

SCIENCE | 1969年 / 163卷 / 3870期

关键词：

D O I：

10.1126/science.163.3870.946

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

We have demonstrated a tenfold (or greater) reduction of the cleavage of galactose from a mucopolysaccharide and a glycoprotein in generalized gangliosidosis, a lysosomal β-galactosidase deficiency disease. The bony abnormalities in this disease may be due to defective catabolism of mucopolysaccharides in connective tissue.

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页码：946 / &

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