TARDBP and amyotrophic lateral sclerosis: In this study, we showed that aggregate-prone TDP-43 truncation mutants increased the TNF-α-mediated NF-κB activity, and this was suppressed by HOIPIN-8 (Figure 7), suggesting that LUBAC activity suppression by HOIPIN-8 or its derivatives will be a potential therapeutic target to suppress ALS-mediated neuroinflammation.