Misfolded SOD1 is able to activate microglia by binding to the CD14/TLR4 receptor (Beers et al., 2008) and expression of TDP-43 increases pro-inflammatory markers, like IL-6, and TNFa which in glial and neuronal cells from ALS patients can act as co-activators of NF-κB (Swarup et al., 2011). Here, TARDBP is linked to amyotrophic lateral sclerosis.