It should also be pointed out that activation of microglial NLRP3 inflammasome with TAR DNA-binding protein 43 (TDP43)—the major pathological protein in sporadic ALS and the closely related frontotemporal dementia—was shown to be toxic to motoneurons in a microglia-motoneuron co-culture [128]. Here, TARDBP is linked to frontotemporal dementia.