However, independent of C9-ALS/FTD, the inhibition of the 26S proteasome has also been shown to lead to the cytoplasmic mislocalization and aggregation of TAR DNA-binding protein 43 (TDP43) [38,39], a second aggregation-prone protein associated with the vast majority of familiar and sporadic forms of ALS. The gene discussed is TARDBP; the disease is frontotemporal dementia.