Gerstmann-sträussler-scheinker disease and anchorless prion protein mice share prion conformational properties diverging from sporadic creutzfeldt-jakob disease

Gianluigi Zanusso, Michele Fiorini, Sergio Ferrari, Kimberly Meade-White, Ilaria Barbieri, Emiliana Brocchi, Bernardino Ghetti, Salvatore Monaco

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Background: Prion strains exhibit distinct physical and biochemical repertoires, aggregation propensity, and biological properties. Results: A biochemical approach is developed for defining the conformational features of prions with or without glycosylphosphatidylinositol (GPI)-anchor. Conclusion: GPI anchorless prions are detected in human genetic prion diseases, but not in sporadic forms. Significance: Unveiling the structure of GPI anchorless prions to predict pathological properties.

Original languageEnglish
Pages (from-to)4870-4881
Number of pages12
JournalJournal of Biological Chemistry
Volume289
Issue number8
DOIs
StatePublished - Feb 21 2014

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Prion Diseases
Prions
Glycosylphosphatidylinositols
Inborn Genetic Diseases
Medical Genetics
Agglomeration
Mouse Prnp protein
Sporadic Creutzfeldt-Jakob Disease

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Cite this

Gerstmann-sträussler-scheinker disease and anchorless prion protein mice share prion conformational properties diverging from sporadic creutzfeldt-jakob disease. / Zanusso, Gianluigi; Fiorini, Michele; Ferrari, Sergio; Meade-White, Kimberly; Barbieri, Ilaria; Brocchi, Emiliana; Ghetti, Bernardino; Monaco, Salvatore.

In: Journal of Biological Chemistry, Vol. 289, No. 8, 21.02.2014, p. 4870-4881.

Research output: Contribution to journalArticle

Zanusso, Gianluigi ; Fiorini, Michele ; Ferrari, Sergio ; Meade-White, Kimberly ; Barbieri, Ilaria ; Brocchi, Emiliana ; Ghetti, Bernardino ; Monaco, Salvatore. / Gerstmann-sträussler-scheinker disease and anchorless prion protein mice share prion conformational properties diverging from sporadic creutzfeldt-jakob disease. In: Journal of Biological Chemistry. 2014 ; Vol. 289, No. 8. pp. 4870-4881.
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AU - Ghetti, Bernardino

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