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 journalArticle

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

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Creutzfeldt-Jakob Syndrome
Brain
Protein Isoforms
Peptide Hydrolases
Proteins
Sporadic Creutzfeldt-Jakob Disease

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Sporadic Creutzfeldt-Jakob disease : Co-occurrence of different types of PrP(Sc) in the same brain. / Puoti, G.; Giaccone, G.; Rossi, G.; Canciani, B.; Bugiani, O.; Tagliavini, Fabrizio.

In: Neurology, Vol. 53, No. 9, 10.12.1999, p. 2173-2176.

Research output: Contribution to journalArticle

Puoti, G. ; Giaccone, G. ; Rossi, G. ; Canciani, B. ; Bugiani, O. ; Tagliavini, Fabrizio. / Sporadic Creutzfeldt-Jakob disease : Co-occurrence of different types of PrP(Sc) in the same brain. In: Neurology. 1999 ; Vol. 53, No. 9. pp. 2173-2176.
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