BIOCHEMICAL BASIS OF LATE-ONSET NEUROLIPIDOSES

被引：45

作者：

CONZELMANN, E ^{[1
]}

SANDHOFF, K ^{[1
]}

机构：

[1] UNIV BONN,INST ORGAN CHEM & BIOCHEM,W-5300 BONN,GERMANY

来源：

DEVELOPMENTAL NEUROSCIENCE | 1991年 / 13卷 / 4-5期

关键词：

LYSOSOMES; STORAGE DISEASES; NEUROLIPIDOSIS; GANGLIOSIDOSIS; METACHROMATIC LEUKODYSTROPHY; RESIDUAL ACTIVITY; LATE ONSET;

D O I：

10.1159/000112160

中图分类号：

Q [生物科学];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

The occurrence and the tremendous phenotypic variability of late-onset neurolipidosis variants are explained on the basis of a simple kinetic model that describes the correlation between residual activity of a deficient lysosomal enzyme and the degradation rate of its substrate in the lysosome.

引用

页码：197 / 204

页数：8

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