The Open UniversitySkip to content
 

Microglia and the pathogenesis of spongiform encephalopathies

Rezaie, P. and Lantos, P.L. (2001). Microglia and the pathogenesis of spongiform encephalopathies. Brain Research Reviews, 35(1) pp. 55–72.

Warning

This is the latest version of this eprint.

DOI (Digital Object Identifier) Link: http://dx.doi.org/10.1016/S0165-0173(01)00042-X
Google Scholar: Look up in Google Scholar

Abstract

Alterations in the phenotype and function of microglia, the resident mononuclear phagocytes of the central nervous system, are among the earliest indications of pathology within the brain and spinal cord. The prion diseases, also known as spongiform encephalopathies, are fatal neurodegenerative disorders with sporadic, genetic or acquired infectious manifestations. A hallmark of all prion diseases is the aberrant metabolism and resulting accumulation of the prion protein. Conversion of the normal cellular protein [PrP(c)] into the abnormal pathogenic (or disease-causing) isoform [PrP(Sc)] involves a conformational alteration whereby the alpha-helical content is transformed into beta-sheet. The histological characteristics of these disorders are spongiform change, astrocytosis, neuronal loss and progressive accumulation of the protease-resistant prion isoform. An additional upregulation in microglial response has been reported in Kuru, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome (GSS), scrapie, in transgenic murine models and in culture, where microglial activation often accompanies prion protein deposition and neuronal loss. This article will review the roles of microglia in spongiform encephalopathies.

Item Type: Journal Article
ISSN: 0165-0173
Academic Unit/Department: Science > Life, Health and Chemical Sciences
Interdisciplinary Research Centre: Biomedical Research Network (BRN)
Item ID: 2573
Depositing User: Payam Rezaie
Date Deposited: 14 Jun 2006
Last Modified: 10 Mar 2014 09:26
URI: http://oro.open.ac.uk/id/eprint/2573
Share this page:

Available Versions of this Item

Altmetrics

Scopus Citations

Actions (login may be required)

View Item
Report issue / request change

Policies | Disclaimer

© The Open University   + 44 (0)870 333 4340   general-enquiries@open.ac.uk