TARDBP and amyotrophic lateral sclerosis: SOD1-ALS seems to be a distinct form of ALS, since the neuropathological hallmark in ~95% of ALS cases is the cytoplasmic mislocalization and aggregation of hyperphosphorylated TDP-43 (pTDP-43) within neurons and glia, while in SOD1-ALS, no pTDP43 staining is detected in the motor cortex [4].