The gangliosidoses: Comparative features and research applications

H. J. Baker, G. D. Reynolds, S. U. Walkley, N. R. Cox, G. H. Baker

Research output: Contribution to journalArticle

42 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)635-649
Number of pages15
JournalVeterinary Pathology
Volume16
Issue number6
DOIs
StatePublished - Jan 1 1979
Externally publishedYes

ASJC Scopus subject areas

  • veterinary(all)

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