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Exp Neurol. 2014 Dec;262 Pt B:111-20. doi: 10.1016/j.expneurol.2014.05.015. Epub 2014 May 22.

Glial cells in amyotrophic lateral sclerosis.

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  • 1Johns Hopkins University, Baltimore, MD, USA.


For more than twenty years glial cells have been implicated in the pathogenetic cascades for genetic and sporadic forms of ALS. The biological role of glia, including the principal CNS glia, astroglia and oligodendroglia, as well as the myeloid derived microglia, has uniformly led to converging data sets that implicate these diverse cells in the degeneration of neurons in ALS. Originating as studies in postmortem human brain implicating astroglia, the research progressed to strongly implicate microglia and contributors to CNS injury in all forms of ALS. Most recently and unexpectedly, oligodendroglia have also been shown in animal model systems and human brain to play an early role in the dysfunction and death of ALS neurons. These studies have identified a number of diverse cellular cascades that could be, or have already been, the target of therapeutic interventions. Understanding the temporal and regional role of these cells and the magnitude of their contribution will be important for future interventions. Employing markers of these cell types may also allow for future important patient subgrouping and pharmacodynamic drug development tools.


Astroglia; C9ORF72; EAAT2; Excitotoxicity; Inflammation; MCT1; Microglia; Oligodendroglia; RNA; Transporters

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