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Related Concept Videos

Amyloid Fibrils03:03

Amyloid Fibrils

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Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining,...
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Author Spotlight: Insight Into Advances in Prion Diseases Research
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Microglia in prion diseases.

Adriano Aguzzi, Caihong Zhu

    The Journal of Clinical Investigation
    |July 18, 2017
    PubMed
    Summary
    This summary is machine-generated.

    Microglia, immune cells in the brain, may protect against prion diseases by clearing harmful proteins. Understanding their activation pathways is key for developing new neurodegenerative disorder treatments.

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    Area of Science:

    • Neuroscience
    • Immunology
    • Pathology

    Background:

    • Prion diseases are fatal neurodegenerative disorders caused by misfolded prion proteins (PrPSc) in the central nervous system (CNS).
    • Microglial activation is a hallmark of prion disease, alongside neuronal loss and spongiform changes.
    • The precise molecular mechanisms of microglial response to prion infection remain largely unknown.

    Purpose of the Study:

    • To investigate the neuroprotective role of microglia in prion pathogenesis.
    • To explore the involvement of microglia-related molecules in prion disease.
    • To understand the regulatory network of microglial activation in prion infection.

    Main Methods:

    • Review of existing literature on microglia and prion disease.
    • Analysis of molecular pathways involved in microglial activation (e.g., TLRs, complement system, cytokines).
    • Discussion of potential therapeutic strategies targeting microglial function.

    Main Results:

    • Evidence suggests microglia may play an overall neuroprotective role in prion pathogenesis.
    • Various microglial molecules are implicated in prion disease progression.
    • A comprehensive understanding of microglial activation regulatory networks is lacking.

    Conclusions:

    • Modulating microglial activation presents a promising therapeutic avenue for prion diseases.
    • Research into microglia in prion disease can enhance understanding of neuroinflammation in other neurodegenerative disorders.