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

G. Puoti, G. Giaccone, G. Rossi, B. Canciani, O. Bugiani, Fabrizio Tagliavini

Research output: Contribution to journalArticlepeer-review

Abstract

Phenotypic heterogeneity of sporadic Creutzfeldt-Jakob disease (CJD) has been linked to biochemically distinct types of the protease-resistant form of the priori protein (type 1 and type 2 prp(Sc)). We investigated 14 cases of sporadic CJD and found that both type I 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.

Original languageEnglish
Pages (from-to)2173-2176
Number of pages4
JournalNeurology
Volume53
Issue number9
Publication statusPublished - Dec 10 1999

Keywords

  • Creutzfeldt-Jakob disease
  • Neuropathology
  • Prion protein
  • Western blot analysis

ASJC Scopus subject areas

  • Neuroscience(all)

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