Recently, it was identified that TBK1 is hyperphosphorylated and sequestered within PolyGA aggregates, leading to the loss of TBK1 activity, enhancing TARDBP aggregation and triggering neurodegeneration in C9ORF72 ALS-FTD (Shao et al., 2022). Here, TARDBP is linked to amyotrophic lateral sclerosis.