Inclusions of protein aggregates containing the TAR-DNA binding protein-43 (TDP43) are pathogenic hallmarks in the vast majority of sporadic cases of Amyotrophic Lateral Sclerosis (ALS) and in a substantial proportion of Frontotemporal Lobar Degeneration (FTLD) [1–4]. The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.