Further evidence that necroptosis may be involved in ALS neurodegeneration comes from in vivo studies in which the administration of the small molecule Nec-1s, a kinase antagonist of the receptor-interacting protein kinase 1 (RIPK1), or deletion of RIPK3, two key determinants of necroptosis (Grootjans et al., 2017), was shown to delay the onset of the ALS phenotype in Tg SOD1G93A mice (Ito et al., 2016). Here, RIPK3 is linked to amyotrophic lateral sclerosis.