C9orf72 knockout myeloid cells are selectively hyperresponsive to activators of STING indicating that STING hyper-activation may be a cause of ALS and FTD.411 TDP-43 aggregates can also mislocate into mitochondria and cause mtDNA release, leading to STING dependent upregulation of inflammatory cytokines.81 This evidence concerns the gene TARDBP and amyotrophic lateral sclerosis.