Sporadic Creutzfeldt-Jakob disease: Co-occurrence of different types of PrP Sc in the same brain
- 1 December 1999
- journal article
- Published by Wolters Kluwer Health in Neurology
- Vol. 53 (9) , 2173
- https://doi.org/10.1212/wnl.53.9.2173
Abstract
Article abstract Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the protease-resistant form of the prion protein (type 1 and type 2 PrPSc). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrPSc coexisted in 5 subjects. The distinct PrPSc isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrPSc type plays a central role in determining the neuropathologic profile of CJD.Keywords
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