The ε isoform of 14-3-3 protein is a component of the prion protein amyloid deposits of Gerstmann-Sträussler-Scheinker disease

Giuseppe Di Fede, Giorgio Giaccone, Lucia Limido, Michela Mangieri, Silvia Suardi, Gianfranco Puoti, Michela Morbin, Giulia Mazzoleni, Bernardino Ghetti, Fabrizio Tagliavini

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

The 14-3-3 proteins are highly conserved, ubiquitous molecules involved in a variety of biologic events, such as transduction pathway modulation, cell cycle control, and apoptosis. Seven isoforms have been identified that are abundant in the brain, preferentially localized in neurons. Remarkable increases in 14-3-3 are seen in the cerebrospinal fluid of patients with Creutzfeldt-Jakob disease (CJD), and it has been found in pathologic inclusions of several neurodegenerative diseases. Moreover, the ζ isoform has been detected in prion protein (PrP) amyloid deposits of CJD patients. To further investigate the cerebral distribution of 14-3-3 in prion-related encephalopathies, we carried out an immunohistochemical and biochemical analysis of brain tissue from patients with Gerstmann-Sträussler-Scheinker disease (GSS) and sporadic, familial and acquired forms of CJD, using specific antibodies against the seven 14-3-3 isoforms. The study showed a strong immunoreactivity of PrP amyloid plaques of GSS patients for the 14-3-3 ε isoform, but not for the other isoforms. The ε isoform of 14-3-3 was not found in PrP deposits of CJD. These results indicate that the ε isoform of 14-3-3 is a component of PrP amyloid deposits of GSS and suggest that this is the sole 14-3-3 isoform specifically involved in the neuropathologic changes associated with this disorder.

Original languageEnglish (US)
Pages (from-to)124-130
Number of pages7
JournalJournal of Neuropathology and Experimental Neurology
Volume66
Issue number2
DOIs
StatePublished - Feb 2007

Keywords

  • 14-3-3
  • Amyloid
  • Creutzfeldt-Jakob disease
  • Gerstmann-Sträussler- Scheinker disease
  • Prion protein

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neuroscience(all)

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