Neuronal GM1 gangliosidosis in a Siamese cat with beta-galactosidase deficiency.
A juvenile Siamese cat with severe, progressive motor disability was shown to have extensive neuronal degeneration caused by accumulation of GM(1) ganglioside. Tissues from brain and kidney were markedly deficient in beta-galactosidase activity. The disease in this cat is thought to be inherited as an autosomal recessive trait, and is strikingly similar to juvenile GM(1) gangliosidosis of children.
D000818:Animals; D001921:Brain / Q000201:enzymology; D001923:Brain Chemistry; D002371:Cat Diseases / Q000378:metabolism*; D002415:Cats; D002531:Cerebellum / Q000473:pathology; D002855:Chromatography, Thin Layer; D005696:Galactosidases / Q000032:analysis*; D005732:Gangliosides / Q000032:analysis; D006801:Humans; D007668:Kidney / Q000201:enzymology; D008052:Lipid Metabolism, Inborn Errors / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000662:veterinary*; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D010375:Pedigree; D011689:Purkinje Cells; D013429:Sulfatases / Q000032:analysis
D005732:Gangliosides; D013429:Sulfatases; D005696:Galactosidases