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 PrP(Sc)). We investigated 14 cases of sporadic CJD and found that both type 1 and type 2 PrP(Sc) coexisted in 5 subjects. The distinct PrP(Sc) isoforms were associated with different patterns of PrP deposition and severity of spongiform changes, suggesting that the PrP(Sc) type plays a central role in determining the neuropathologic profile of CJD.
journal_name
Neurologyjournal_title
Neurologyauthors
Puoti G,Giaccone G,Rossi G,Canciani B,Bugiani O,Tagliavini Fdoi
10.1212/wnl.53.9.2173subject
Has Abstractpub_date
1999-12-10 00:00:00pages
2173-6issue
9eissn
0028-3878issn
1526-632Xjournal_volume
53pub_type
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