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. Copyright

Sporadic Creutzfeldt-Jakob disease : the extent of microglia activation is dependent on the biochemical type of PrPSc / G. Puoti, G. Giaccone, M. Mangieri, L. Limido, P. Fociani, P. Zerbi, S. Suardi, G. Rossi, S. Iussich, R. Capobianco, G. Di Fede, G. Marcon, R. Cotrufo, G. Filippini, O. Bugiani, F. Tagliavini. - In: JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY. - ISSN 0022-3069. - 64:10(2005), pp. 902-909.

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

P. Zerbi;
2005

Abstract

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. Copyright
Creutzfeldt-Jakob disease; Immunohistochemistry; Microglia; Prion diseases; Prion protein; Western blot analysis
2005
http://journals.lww.com/jneuropath/Abstract/2005/10000/Sporadic_Creutzfeldt_Jakob_Disease__The_Extent_of.9.aspx
Article (author)
File in questo prodotto:
Non ci sono file associati a questo prodotto.
Pubblicazioni consigliate

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/16013
Citazioni
  • ???jsp.display-item.citation.pmc??? 16
  • Scopus 32
  • ???jsp.display-item.citation.isi??? 28
social impact