Bovine GM1 gangliosidosis: An inborn lysosomal disease

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The results of studies on GM1 gangliosidosis of Friesian calves are reviewed in the light of present knowledge about inborn lysosomal diseases. Affected calves have poor growth rates and develop signs of incoordination and blindness at about three months of age. The disease is progressive resulting in death at six to nine months of age. Storage of GM1 ganglioside in neurons is associated with reduced activity of tissue β-galactosidase. Evidence is presented suggesting an autosomal recessive mode of inheritance. The implications of these findings relative to diagnosis and control are discussed.