Moreover, astroglia were derived from iPSCs obtained from an ALS patient carrying the TARDBP mutation in order to investigate the suspected role of glial cell activation in ALS pathogenesis. These derived astroglia showed TDP-43 (the protein product) proteinopathy, such as mislocalization of TDP-43, increased total cellular levels of TDP-43, and decreased cell survival. However, upon co-culture of the derived astroglia with derived motor neurons from the same iPSCs of the ALS patient or from control normal patients, there were no effects of any kind on the neurons in culture suggesting other involved mechanisms as previously described for SOD1 ALS (Serio et al., 2013). In this context, motor neurons were successfully differentiated from iPSCs of an 82 year old patient with familial ALS of this same TDP-43 mutation (Dimos et al., 2008). Besides, iPSCs have been derived from ALS patients carrying the C9ORF72 mutation which is also involved in frontotemporal dementia and these have also uncovered some characteristic phenotypes in ALS (Donnelly et al., 2013; Sareen et al., 2013; Hedges et al., 2016). All this further supports the notion of iPSCs'
