Thus, TDP-43 deficiency, as it may occur in human disease by cytosolic sequestration of this nuclear protein [26], causes a neurite defect (in addition to deregulated aggregating toxic protein turnover [11]) that may contribute to motorneuron disease in ALS, and if occurring in the frontal and temporal cortex also to FTLD-TDP. Here, TARDBP is linked to amyotrophic lateral sclerosis.