Sporadic Creutzfeldt-Jakob disease: The extent of microglia activation is dependent on the biochemical type of PrPSc

Gianfranco Puoti, Giorgio Giaccone, Michela Mangieri, Lucia Limido, Paolo Fociani, Pietro Zerbi, Silvia Suardi, Giacomina Rossi, Selina Iussich, Raffaella Capobianco, Giuseppe Di Fede, Gabriella Marcon, Roberto Cotrufo, Graziella Filippini, Orso Bugiani, Fabrizio Tagliavini

Research output: Contribution to journalArticlepeer-review


In prion-related encephalopathies, microglial activation occurs early and is dependent on accumulation of disease-specific forms of the prion protein (PrPSc) and may play a role in nerve cell death. Previously, we found that different types of PrPSc (i.e. type 1 and type 2) coexisted in approximately 25% of patients with sporadic Creutzfeldt-Jakob disease (CJD); and a close relationship was detected between PrPSc type, the pattern of PrP immunoreactivity, and extent of spongiform degeneration. To investigate whether microglial reaction is related to the biochemical type and deposition pattern of PrPSc, we carried out a neuropathologic and biochemical study on 26 patients with sporadic CJD, including all possible genotypes at codon 129 of the prion protein gene. By quantitative analysis, we demonstrated that strong microglial activation was associated with type 1 PrPSc and diffuse PrP immunoreactivity, whereas type 2 PrPSc and focal PrP deposits were accompanied by mild microglia reaction. These findings support the view that the phenotypic heterogeneity of sporadic CJD is largely determined by the physicochemical properties of distinct PrPSc conformers.

Original languageEnglish
Pages (from-to)902-909
Number of pages8
JournalJournal of Neuropathology and Experimental Neurology
Issue number10
Publication statusPublished - Oct 2005


  • Creutzfeldt-Jakob disease
  • Immunohistochemistry
  • Microglia
  • Prion diseases
  • Prion protein
  • Western blot analysis

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Neuroscience(all)


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