GLYCOLIPID AND MUCOPOLYSACCHARIDE ABNORMALITY IN FIBROBLASTS OF FABRYS DISEASE

被引：49

作者：

MATALON, R

DORFMAN, A

DAWSON, G

SWEELEY, CC

机构：

[1] Departments of Pediatrics and Biochemistry, Mental Retardation Center, University of Chicago, Chicago, IL

[2] Department of Biochemistry, Michigan State University, East Lansing, MI

来源：

SCIENCE | 1969年 / 164卷 / 3887期

关键词：

D O I：

10.1126/science.164.3887.1522

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：

Cultures of skin fiibroblasts from a patient with Fabry's disease showed an accumulation of the glycolipid, galactosyl-galactosyl-glucosyl ceramide. Such cells also showed metachromasia on staining with toluidine blue and a markedly elevated acid mucopolysaccharide content.

引用

页码：1522 / &

共 21 条

[1] ENZYMATIC DEFECT IN FABRYS DISEASE - CERAMIDETRIHEXOSIDASE DEFICIENCY [J].