The gangliosidoses

Comparative features and research applications

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

Research output: Contribution to journalArticle

41 Citations (Scopus)

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
StatePublished - 1979
Externally publishedYes

Fingerprint

Gangliosidoses
gangliosides
Gangliosides
enzyme deficiencies
purebreds
hydrolases
hypertrophy
Research
domestic animals
neurons
deterioration
Domestic Animals
Hydrolases
cats
metabolites
Hypertrophy
swine
dogs
cattle
Cats

ASJC Scopus subject areas

  • veterinary(all)

Cite this

Baker, H. J., Reynolds, G. D., Walkley, S. U., Cox, N. R., & Baker, G. H. (1979). The gangliosidoses: Comparative features and research applications. Veterinary Pathology, 16(6), 635-649.

The gangliosidoses : Comparative features and research applications. / Baker, H. J.; Reynolds, G. D.; Walkley, Steven U.; Cox, N. R.; Baker, G. H.

In: Veterinary Pathology, Vol. 16, No. 6, 1979, p. 635-649.

Research output: Contribution to journalArticle

Baker, HJ, Reynolds, GD, Walkley, SU, Cox, NR & Baker, GH 1979, 'The gangliosidoses: Comparative features and research applications', Veterinary Pathology, vol. 16, no. 6, pp. 635-649.
Baker, H. J. ; Reynolds, G. D. ; Walkley, Steven U. ; Cox, N. R. ; Baker, G. H. / The gangliosidoses : Comparative features and research applications. In: Veterinary Pathology. 1979 ; Vol. 16, No. 6. pp. 635-649.
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