Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the proteaseresistant 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.
Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain
PUOTI, Gianfranco;
1999
Abstract
Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the proteaseresistant 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.File in questo prodotto:
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