TARDBP and amyotrophic lateral sclerosis: In contrast, inhibition of STING using a validated STING inhibitor, H-151 [95] in ALS patient derived iPSCs and a TDP-43 mouse model normalises IFN levels, resulting in reduced neuronal loss and improved motor performance in mice [86], providing further evidence for the role of inflammation in the propagation of neurodegeneration in ALS (Fig. 2).