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

Shi Q, Xie WL, Zhang B, Chen LN, Xu Y, Wang K, Ren K, Zhang XM, Chen C, Zhang J, Dong XP - Virol. J. (2013)

Bottom Line: However, the situation of microglia in other genetic prion diseases such as fatal familial insomnia (FFI) and familial CJD remains less understood.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: State Key Laboratory for Infectious Disease Prevention and Control, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Hangzhou), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Chang-Bai Rd 155, Beijing 102206, People's Republic of China.

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.

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Related in: MedlinePlus

Immunofluorescent assays of microglia (with Iba-1 specific antibody) and PrPSc (with PrP specific antibody) in the temporal lobes of different human prion diseases. The images of PrPSc (green), Iba-1 (red), DAPI (blue) and merge are monitored under a confocal microscopy and indicated above. Various prion diseases and normal control are indicated on the left.
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Figure 4: Immunofluorescent assays of microglia (with Iba-1 specific antibody) and PrPSc (with PrP specific antibody) in the temporal lobes of different human prion diseases. The images of PrPSc (green), Iba-1 (red), DAPI (blue) and merge are monitored under a confocal microscopy and indicated above. Various prion diseases and normal control are indicated on the left.

Mentions: Furthermore, Iba1- and PrP-specific double-stained immunofluorescent assays were conducted with the brain sections of various human prion diseases after treatment with GdnHCl. Confocal microscopy illustrated more PrPSc specific signals (green) in the brain tissues of sCJD cases, less amounts in G114V gCJD, whereas almost undetectable in FFI cases (Figure 4) Meanwhile, obviously large amounts of Iba1 specific signals (red) were observed in the brain sections of sCJD cases, but clearly less in that of FFI cases and G114V gCJD (Figure 4), which highlights an active proliferation of microglia specially in the brain tissues of sCJD.


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

Shi Q, Xie WL, Zhang B, Chen LN, Xu Y, Wang K, Ren K, Zhang XM, Chen C, Zhang J, Dong XP - Virol. J. (2013)

Immunofluorescent assays of microglia (with Iba-1 specific antibody) and PrPSc (with PrP specific antibody) in the temporal lobes of different human prion diseases. The images of PrPSc (green), Iba-1 (red), DAPI (blue) and merge are monitored under a confocal microscopy and indicated above. Various prion diseases and normal control are indicated on the left.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3716817&req=5

Figure 4: Immunofluorescent assays of microglia (with Iba-1 specific antibody) and PrPSc (with PrP specific antibody) in the temporal lobes of different human prion diseases. The images of PrPSc (green), Iba-1 (red), DAPI (blue) and merge are monitored under a confocal microscopy and indicated above. Various prion diseases and normal control are indicated on the left.
Mentions: Furthermore, Iba1- and PrP-specific double-stained immunofluorescent assays were conducted with the brain sections of various human prion diseases after treatment with GdnHCl. Confocal microscopy illustrated more PrPSc specific signals (green) in the brain tissues of sCJD cases, less amounts in G114V gCJD, whereas almost undetectable in FFI cases (Figure 4) Meanwhile, obviously large amounts of Iba1 specific signals (red) were observed in the brain sections of sCJD cases, but clearly less in that of FFI cases and G114V gCJD (Figure 4), which highlights an active proliferation of microglia specially in the brain tissues of sCJD.

Bottom Line: However, the situation of microglia in other genetic prion diseases such as fatal familial insomnia (FFI) and familial CJD remains less understood.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: State Key Laboratory for Infectious Disease Prevention and Control, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases (Hangzhou), National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Chang-Bai Rd 155, Beijing 102206, People's Republic of China.

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.

Show MeSH
Related in: MedlinePlus