A lysosomal storage disease of Romney sheep that resembles human type 3 G(M1) gangliosidosis

During a large neuropathological survey of clinically normal sheep for evid ence of scrapie, three adult Romney ewes from the same farm were found to h ave populations of distended neurons containing granular eosinophilic stora ge material. The affected neurons were confined to the striatum. The granul es had the tinctorial properties of glycolipid and the ultrastructural appe arance of spherical bodies containing concentric membranous whorls. The bod ies resembled those of G(M1) gangliosidosis. The restricted neuroanatomical distribution of changes together with the age of the sheep suggests a simi larity to human type 3 G(M1) gangliosidosis, which has not previously been reported in animals.