Loss-of-function of OPTN leads to progressive dysmyelination and axonal degeneration through the engagement of necroptotic machinery in the central nervous system (CNS), including RIPK1, RIPK3, and mixed lineage kinase domain-like protein (MLKL) [120], suggesting a crucial role of OPTN in the sensitization to necroptotic death in ALS pathology. This evidence concerns the gene OPTN and amyotrophic lateral sclerosis.