Neuronal GM 1 Gangliosidosis in a Siamese Cat with β-Galactosidase Deficiency

Abstract

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.