GANGLIOSIDOSES - COMPARATIVE FEATURES AND RESEARCH APPLICATIONS

被引：50

作者：

BAKER, HJ

REYNOLDS, GD

WALKLEY, SU

COX, NR

BAKER, GH

机构：

来源：

VETERINARY PATHOLOGY | 1979年 / 16卷 / 06期

关键词：

D O I：

10.1177/030098587901600602

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

Ganglioside storae diseases are inherited defects of lysosomal hydrolases that result in intralysosomal accumulation of gangliosides and other complex metabolites. Gangliosidoses occur in man, cats, cattle, dogs and swine. In all species, these diseases are characterized clinically by relentlessly progressive neurological deterioration. Lysosomal hypertrophy with characteristic ultrastructural inclusions occur in neurons, endothelial and other cells. Definitive diagnosis requires biochemical identification of the storage product and enzyme deficiency. Gangliosidoses in animals are important models of human lysosomal diseases and may be a significant complication in the maintenance of certain purebred stocks of domestic animals.

引用

页码：635 / 649

页数：15

共 45 条

[1]

BAKER HJ, 1976, FED PROC, V35, P1193

[2] NEURONAL GM1 GANGLIOSIDOSIS IN A SIAMESE CAT WITH BETA-GALACTOSIDASE DEFICIENCY [J].