In line with the genetic aetiology of ALS, CNS tissues from around 97% of ALS patients, including both sporadic and familial cases, display the mislocalization and aggregation of TDP-43 (TDP-43 proteinopathy) (Neumann et al., 2006; Ling et al., 2013). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.