Neuronal-visceral GM1 gangliosidosis in a dog with beta-galactosidase deficiency

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Science  22 Oct 1976:
Vol. 194, Issue 4263, pp. 442-445
DOI: 10.1126/science.824730


A 9-month-old dog with a history of progressive motor dysfunction was shown to have a deficiency in brain beta-galactosidase activity. The canine disease, like that of children with GM1 gangliosidosis, is characterized by accumulation of GM1 ganglioside in the brain, liver, and spleen, and membranous cytoplasmic bodies in neurons. The dog's pedigree suggests an autosomal recessive pattern of inheritance.