In addition to the common TDP-43 aggregates in FTLD and ALS, C9orf72 mutation carriers have abundant star-shaped, TDP-43-negative neuronal cytoplasmic inclusions (NCI) particularly in the cerebellum, hippocampus and frontal neocortex that stain positive for markers of the proteasome system (UPS) such as p62 or ubiquitin [1, 7]. Here, TARDBP is linked to amyotrophic lateral sclerosis.