Canine GM1 gangliosidosis: An Ultrastructural and Biochemical Study

Moses Rodriguez, John S. O'Brien, Robert S. Garrett, Henry C. Powell

Research output: Contribution to journalArticlepeer-review

21 Scopus citations

Abstract

The ultrastructural and biochemical features of canine GM1 gangliosidosis were studied. B-Galactosidase activity assayed using both skin fibroblast tissue culture strains and fresh skin revealed enzyme activities in three groups (normals, heterozygotes, and homozygotes) corresponding to an autosomal recessive inheritance. The concentration of ganglioside GM1 was greatly increased in cerebral gray matter and kidney. A striking elevation of tissue oligosaccharides was found in liver, kidney, and spleen. Most neurons in the cerebral cortex and deep gray matter were filled by spherical lamellated inclusions. Hepatocytes contained vacuoles with an amorphous granular material which may correspond to the accumulation of galactose-oligosac-charides determined chemically. The disease in dogs has features similar to both the infantile and juvenile form of human GM1 gangliosidosis.

Original languageEnglish (US)
Pages (from-to)618-629
Number of pages12
JournalJournal of Neuropathology and Experimental Neurology
Volume41
Issue number6
DOIs
StatePublished - Nov 1982

Keywords

  • B-galactosidase
  • Dog diseases
  • Electron
  • Gangliosides
  • Microscopy
  • Pathology
  • Veterinary

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neurology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience

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