Brain microglia were activated in sporadic CJD but almost unchanged in fatal familial insomnia and G114V genetic CJD.

Abstract

BACKGROUND:

Microglial activations have been described in different subtypes of human prion diseases such as sporadic Creutzfeldt-Jakob disease (CJD), variant CJD, Kuru and Gerstmann-Sträussler-Scheinker disease (GSS). However, the situation of microglia in other genetic prion diseases such as fatal familial insomnia (FFI) and familial CJD remains less understood. The brain microglia was evaluated comparatively between the FFI, G114V and sCJD cases in the study.

METHODS:

Specific Western blots, immunohistochemical and immunofluorescent assays were used to detect the changes of microglia and ELISA tests were used for levels of inflammatory cytokines.

RESULTS:

Western blots, immunohistochemical and immunofluorescent assays illustrated almost unchanged microglia in the temporal lobes of FFI and G114V gCJD, but obviously increased in those of sCJD. The Iba1-levels maintained comparable in six different brain regions of FFI and G114V cases, including thalamus, cingulate gyrus, frontal cortex, parietal cortex, occipital cortex and temporal cortex. ELISA tests for inflammatory cytokines revealed significantly up-regulated IL-1β, IL-6 and TNF-α in the brain homogenates from sCJD, but not in those from FFI and G114V gCJD.

CONCLUSION:

Data here demonstrates silent brain microglia in FFI and G114V gCJD but obviously increased in sCJD, which reflects various pathogenesis of different human prion diseases subtypes.

• Authors: Qi Shi, Wu-Ling Xie, Baoyun Zhang, Li-Na Chen, Yin Xu, Ke Wang, Ke Ren, Xiao-Mei Zhang, Cao Chen, Jin Zhang, Xiao-Ping Dong
• Journal: Virology journal (Virol J) Vol. 10 Pg. 216 (Jul 01 2013) ISSN: 1743-422X [Electronic] England
• PMID: 23816234 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Cytokines

Topics

• Blotting, Western
• Brain (pathology)
• Creutzfeldt-Jakob Syndrome (immunology, pathology)
• Cytokines (metabolism)
• Encephalopathy, Bovine Spongiform (immunology, pathology)
• Enzyme-Linked Immunosorbent Assay
• Humans
• Immunohistochemistry
• Insomnia, Fatal Familial (immunology, pathology)
• Microglia (immunology)
• Microscopy, Fluorescence