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From: TSS ()
Subject: sCJD: The Extent of Microglia Activation Is Dependent on the Biochemical Type of PrPSc
Date: October 11, 2005 at 2:48 pm PST

##################### Bovine Spongiform Encephalopathy #####################


sCJD: The Extent of Microglia Activation Is Dependent on the
Tue Oct 11, 2005 16:36
70.110.83.173

Sporadic Creutzfeldt-Jakob Disease: The Extent of Microglia Activation Is Dependent on the Biochemical Type of PrPSc.
Journal of Neuropathology & Experimental Neurology. 64(10):902-909, October 2005.
Puoti, Gianfranco MD, PhD; Giaccone, Giorgio MD; Mangieri, Michela DVM; Limido, Lucia PhD; Fociani, Paolo MD; Zerbi, Pietro MD; Suardi, Silvia PhD; Rossi, Giacomina PhD; Iussich, Selina DVM; Capobianco, Raffaella DVM; Di Fede, Giuseppe MD, PhD; Marcon, Gabriella MD; Cotrufo, Roberto MD; Filippini, Graziella MD; Bugiani, Orso MD; Tagliavini, Fabrizio MD
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.

(C) 2005 American Association of Neuropathologists, Inc

http://www.jneuropath.com/pt/re/jnen/abstract.00005072-200510000-00009.htm;jsessionid=DMtnIWUn96qXEOCLOnxxqId8RosR7pAEOV6DN1gPliFYMJmAfx6O!-365670234!-949856145!9001!-1

TSS

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