In the context of ALS/FTD, Tnks-1/2 inhibitors, and potentially inhibitors specific for the TDP-43 and Tnks-1/2 interaction, could help maintain TDP-43 in the nucleus, where damaged and misfolded forms of the protein can be turned over by the proteasome. This evidence concerns the gene TNKS and amyotrophic lateral sclerosis.