While aggregates of SOD1 and FUS are mostly limited to patients with mutations in the corresponding genes, aberrantly distributed and aggregated TDP-43 is additionally evident in patients with mutations in other genes including C9ORF72 as well as SALS, further pronouncing the central role of TDP-43 in ALS [1,9,10]. Here, C9orf72 is linked to amyotrophic lateral sclerosis.