Finally, inhibiting SG accumulation by targeting eIF2α phosphorylation, one of the most studied drivers for SG formation, or key SG proteins such as TDP-43, Ataxin 2, or TIA1 protects against disease-associated phenotypes in neuron culture or animal models of ALS/FTD12,19–22. This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.