Neuronal GM1 Gangliosidosis in a Siamese Cat with β-Galactosidase Deficiency

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Science  19 Nov 1971:
Vol. 174, Issue 4011, pp. 838-839
DOI: 10.1126/science.174.4011.838


A juvenile Siamese cat with severe, progressive motor disability was shown to have extensive neuronal degeneration caused by accumulation of GM1 ganglioside. Tissues from brain and kidney were markedly deficient in β-galactosidase activity. The disease in this cat is thought to be inherited as an autosomal recessive trait, and is strikingly similar to juvenile GM1 gangliosidosis of children.