AUTHOR=Radford Rowan A. , Morsch Marco , Rayner Stephanie L. , Cole Nicholas J. , Pountney Dean L. , Chung Roger S. 

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

JOURNAL=Frontiers in Cellular Neuroscience

VOLUME=9

YEAR=2015

URL=https://www.frontiersin.org/journals/cellular-neuroscience/articles/10.3389/fncel.2015.00414

DOI=10.3389/fncel.2015.00414

ISSN=1662-5102

ABSTRACT=<p>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 <italic>vice versa</italic>. Recent genetic discoveries conclusively link the two diseases, and several common molecular players have been identified (<italic>TDP-43, FUS, C9ORF72</italic>). 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.</p>