It is noteworthy that C-terminal truncation of TDP-43 is commonly associated with TDP-43 pathology in the brain, but rarely observed in the spinal cord of individuals with ALS and FTD, suggesting that the molecular composition of pathological TDP-43 inclusions is (to some extent) tissue-specific [71, 72]. Here, TARDBP is linked to amyotrophic lateral sclerosis.