Loss of tdp‐1 protects against the toxicity induced by aggregation‐prone 25‐KDa carboxyl fragment TDP‐C25 [138], a unique component of the TDP‐43 positive inclusions in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) patient brain samples [11, 143]. Here, TARDBP is linked to amyotrophic lateral sclerosis.