Hepatic beta galactosidase and feline GMI gangliosidosis.

Abstract

This paper describes the clinical, morphological and biochemical features of three cats with a progressive neurological disorder. Clinical features were ataxia and progressive tremor. The morphological characteristics were those of lysosomal storage disease affecting neurones of the central nervous system and autonomic ganglia; membranous cytoplasmic bodies were demonstrated by electron microscopy in cerebral neurones. Chemical analysis of brain from two of the cats revealed an increased content of total gangliosides, sialic acid and a specific increase in GMI ganglioside. Enzyme analysis of homogenates of leucocytes, spleen and brain showed less than 5% or normal 4-methylumbelliferyl-beta galactosidase (4MU-beta gal) activity. In liver, activity was markedly reduced at pH values below 4.2, but there was considerable activity above this value. The properties of 4MU-beta gal in normal and diseased feline livers were investigated. Sephadex gel filtration of diseased liver homogenates showed an absence of two thermolabile "acid' components, and reduced activity of a third thermostable "neutral' component. The biochemical abnormalities found in the diseased cats are similar to those found in human juvenile GMI gangliosidosis (type 2).

• Authors: I C Barnes, D F Kelly, C A Pennock, J A Randell
• Journal: Neuropathology and applied neurobiology (Neuropathol Appl Neurobiol) 1981 Nov-Dec Vol. 7 Issue 6 Pg. 463-76 ISSN: 0305-1846 [Print] England
• PMID: 6799849 (Publication Type: Journal Article)

Chemical References

• G(M1) Ganglioside
• Galactosidases
• beta-Galactosidase

Topics

• Animals
• Brain (enzymology, pathology)
• Cat Diseases (enzymology, pathology)
• Cats
• Female
• G(M1) Ganglioside (metabolism)
• Galactosidases (metabolism)
• Gangliosidoses (enzymology, pathology, veterinary)
• Humans
• Liver (enzymology)
• Male
• beta-Galactosidase (metabolism)