The gangliosidoses: Comparative features and research applications

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

doi:10.1177/030098587901600602

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 journal › Article › peer-review

46 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 language	English (US)
Pages (from-to)	635-649
Number of pages	15
Journal	Veterinary Pathology
Volume	16
Issue number	6
DOIs	https://doi.org/10.1177/030098587901600602
State	Published - 1979
Externally published	Yes

ASJC Scopus subject areas

General Veterinary

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10.1177/030098587901600602

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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.",

author = "Baker, {H. J.} and Reynolds, {G. D.} and Walkley, {S. U.} and Cox, {N. R.} and Baker, {G. H.}",

year = "1979",

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AU - Cox, N. R.

AU - Baker, G. H.

PY - 1979

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AB - 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.

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The gangliosidoses: Comparative features and research applications

Abstract

ASJC Scopus subject areas

UN SDGs

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