While it is well recognized that nuclear depletion and cytoplasmic aggregation of TDP-43 do not occur in SOD1 ALS [37], surprisingly we found that 48% of 33 residual motor neurons examined in four SOD1 ALS nervous systems also had nuclear depletion of ELAVL3 (Fig. 3g–i). Here, TARDBP is linked to amyotrophic lateral sclerosis.