In this sense, while the reduction of microgliosis was shown to slow ALS progression in the mutated SOD1 mice (Martínez-Muriana et al., 2016), reactive microglia was protective to MN degeneration in a mouse model of TDP-43 proteinopathy (Spiller et al., 2018), reinforcing the relevance of microglia reactivity and function in the ALS context. Here, SOD1 is linked to amyotrophic lateral sclerosis.