To further characterize ESCRT-III protein pathology and the contribution to NPC injury in familial and sporadic ALS, we now report that VPS4, but not CHMP4B or CHMP2B, is increased in a CHMP7 dependent manner in C9orf72 ALS/FTD and sALS human neuronal nuclei. Here, C9orf72 is linked to frontotemporal dementia.