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  • The established and emerging roles of astrocytes and microglia in amyotrophic lateral sclerosis and frontotemporal dementia

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    Author(s)
    Radford, Rowan A
    Morsch, Marco
    Rayner, Stephanie L
    Cole, Nicholas J
    Pountney, Dean L
    Chung, Roger S
    Griffith University Author(s)
    Pountney, Dean L.
    Year published
    2015
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    Abstract
    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two progressive, fatal neurodegenerative syndromes with considerable clinical, genetic and pathological overlap. Clinical symptoms of FTD can be seen in ALS patients and vice versa. Recent genetic discoveries conclusively link the two diseases, and several common molecular players have been identified (TDP-43, FUS, C9ORF72). The definitive etiologies of ALS and FTD are currently unknown and both disorders lack a cure. Glia, specifically astrocytes and microglia are heavily implicated in the onset and progression of neurodegeneration witnessed in ALS ...
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    Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two progressive, fatal neurodegenerative syndromes with considerable clinical, genetic and pathological overlap. Clinical symptoms of FTD can be seen in ALS patients and vice versa. Recent genetic discoveries conclusively link the two diseases, and several common molecular players have been identified (TDP-43, FUS, C9ORF72). The definitive etiologies of ALS and FTD are currently unknown and both disorders lack a cure. Glia, specifically astrocytes and microglia are heavily implicated in the onset and progression of neurodegeneration witnessed in ALS and FTD. In this review, we summarize the current understanding of the role of microglia and astrocytes involved in ALS and FTD, highlighting their recent implications in neuroinflammation, alterations in waste clearance involving phagocytosis and the newly described glymphatic system, and vascular abnormalities. Elucidating the precise mechanisms of how astrocytes and microglia are involved in ALS and FTD will be crucial in characterizing these two disorders and may represent more effective interventions for disease progression and treatment options in the future.
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    Journal Title
    Frontiers in Cellular Neuroscience
    Volume
    9
    DOI
    https://doi.org/10.3389/fncel.2015.00414
    Copyright Statement
    © 2015 Radford, Morsch, Rayner, Cole, Pountney and Chung. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution and reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
    Subject
    Neurosciences not elsewhere classified
    Biochemistry and Cell Biology
    Neurosciences
    Publication URI
    http://hdl.handle.net/10072/125320
    Collection
    • Journal articles

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