作者: G. Puoti , G. Giaccone , G. Rossi , B. Canciani , O. Bugiani
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摘要: Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types the protease-resistant form prion protein (type 1 and type 2 PrP(Sc)). We investigated 14 cases CJD found that both PrP(Sc) coexisted in 5 subjects. The isoforms were associated with different patterns PrP deposition severity spongiform changes, suggesting plays a central role determining neuropathologic profile CJD.