DEMONSTRATION OF AN ALTERATION OF GANGLIOSIDE METABOLISM IN TAY-SACHS DISEASE

被引：120

作者：

KOLODNY, EH

BRADY, RO

VOLK, BW

机构：

[1] Laboratory of Neurochemistry National Institute of Neurological Diseases, Stroke National Institutes of Health Bethesda

[2] Isaac Albert Research Institute Kingsbrook Jewish Medical Center Brooklyn

来源：

BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS | 1969年 / 37卷 / 03期

关键词：

D O I：

10.1016/0006-291X(69)90947-4

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

It has been shown through the use of specifically labeled Tay-Sachs ganglioside that normal human muscle tissue contains enzymes which catalyze the hydrolysis of the N-acetylneuraminyl and N-acetylgalactosaminyl moieties of this ganglioside. The hydrolysis of the N-acetylneuraminyl moiety in muscle preparations from patients with Tay-Sachs disease was similar to that of the controls. The enzyme which catalyzes the hydrolysis of the N-acetylgalactosaminyl moiety was completely absent in preparations from patients with Tay-Sachs disease. © 1969.

引用

页码：526 / &

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