In the case of TDP-43 in ALS, mitochondrial dysfunction causes inflammation via release of mitochondrial DNA to the cytoplasm, followed by activation of the cGAS/STING-based inflammation pathway; inhibiting STING reduces inflammation and neurodegeneration in in vitro and in vivo models of ALS (Yu et al, 2020). Here, STING1 is linked to amyotrophic lateral sclerosis.