Alpha-synuclein accumulates in the brain of scrapie-affected sheep and goats.

Immunohistochemical examination demonstrated widespread granular deposits of alpha-synuclein (alphaSN) in the brains of sheep and goats with natural scrapie, especially in the cornu ammonis and subiculum of the hippocampus; this contrasted with the diffuse and non-granular immunolabelling seen in healthy controls. There was non-regular "co-localization" of PrP(Sc) and alphaSN. The findings resembled those reported in Creutzfeldt-Jakob disease and in experimental prion disease in hamsters and mice. The results suggest that perturbation of alphaSN metabolism plays a role in human and animal prion diseases.