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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

Immunohistochemical assays of microglia in the temporal lobes of three FFI, two sCJD, the G114V gCJD and normal control with Iba-1 specific antibody. Different prion diseases and normal control are indicated above. Scale bar, 20 μm.
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Figure 3: Immunohistochemical assays of microglia in the temporal lobes of three FFI, two sCJD, the G114V gCJD and normal control with Iba-1 specific antibody. Different prion diseases and normal control are indicated above. Scale bar, 20 μm.

Mentions: To reveal the morphological difference in microglia among various subtypes of human prion diseases, the sections of temporal lobes from three FFI cases, two sCJD cases, one G114V gCJD and a normal control were employed into Iba1 specific IHC assays. Abundances of microglia with much larger round- or amoeboid-shape cell bodies were observed in the brain sections of two sCJDs, but only a few Iba1 positive-stained cells with small cell body were detected in the tested two kinds of inherited prion diseases showing similar patterns as normal one (Figure 3), even in the brains of G114V gCJD which contained lager amounts PrPSc deposits. This kind of enlarged morphology of microglia is usually thought to be associated with an activated and phagocytic state [2], which may suggest an enhanced microglia population in the CNS 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)

Immunohistochemical assays of microglia in the temporal lobes of three FFI, two sCJD, the G114V gCJD and normal control with Iba-1 specific antibody. Different prion diseases and normal control are indicated above. Scale bar, 20 μm.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Immunohistochemical assays of microglia in the temporal lobes of three FFI, two sCJD, the G114V gCJD and normal control with Iba-1 specific antibody. Different prion diseases and normal control are indicated above. Scale bar, 20 μm.
Mentions: To reveal the morphological difference in microglia among various subtypes of human prion diseases, the sections of temporal lobes from three FFI cases, two sCJD cases, one G114V gCJD and a normal control were employed into Iba1 specific IHC assays. Abundances of microglia with much larger round- or amoeboid-shape cell bodies were observed in the brain sections of two sCJDs, but only a few Iba1 positive-stained cells with small cell body were detected in the tested two kinds of inherited prion diseases showing similar patterns as normal one (Figure 3), even in the brains of G114V gCJD which contained lager amounts PrPSc deposits. This kind of enlarged morphology of microglia is usually thought to be associated with an activated and phagocytic state [2], which may suggest an enhanced microglia population in the CNS 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