Prion infection of COCS faithfully reproduced all salient features of the pathogenesis of prion diseases: (1) progressive, profound neuronal loss after a protracted asymptomatic incubation time (42 dpi in tga20 COCS), (2) a proinflammatory glial response with vigorous upregulation of Rantes, MCP-1 and TNFα, (3) typical neuropathological changes such as spongiform changes, tubulovesicular structures, and neuroaxonal dystrophy, and (4) an excessive meshwork of astrocytic processes exceeding that observed in controls and reminiscent of gliosis. This evidence concerns the gene CCL2 and prion disease.