Sj. Ryder et Mm. Simmons, A lysosomal storage disease of Romney sheep that resembles human type 3 G(M1) gangliosidosis, ACT NEUROP, 101(3), 2001, pp. 225-228
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.