Sporadic Creutzfeldt-Jakob disease: co-occurrence of different types of PrP(Sc) in the same brain.

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

Neurology

journal_title

Neurology

authors

Puoti G,Giaccone G,Rossi G,Canciani B,Bugiani O,Tagliavini F

doi

10.1212/wnl.53.9.2173

subject

Has Abstract

pub_date

1999-12-10 00:00:00

pages

2173-6

issue

9

eissn

0028-3878

issn

1526-632X

journal_volume

53

pub_type

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