Nevertheless, recent studies in a mouse model have not supported the hypothesis of Sho protein playing a role as a prion disease modifier, since: a) the down-regulation of Sho and development of clinical prion disease were not synchronized [8], [9]; b) Sho levels following prion infection in transgenic mice overexpressing mouse Sho Tg(MoSho) were higher than those present in uninfected, wt mice [8], [9]; and, c) knocking out Sho protein did not affect the incubation time of the prion-borne disease [11]. This evidence concerns the gene SPRN and prion disease.