Pathologically aggregated TDP-43 is ubiquitinated, hyperphosphorylated, acetylated, and partially cleaved, with several C-terminal fragments (CTFs) with molecular weights of ~ 18–27 kDa forming a major component of phosphorylated TDP-43 (pTDP-43) inclusions in the brain of ALS/FTD patients [138–140, 192]. The gene discussed is TARDBP; the disease is frontotemporal dementia.