Among the aggregated proteins found in motor neurons of ALS patients, TDP-43 is present in about 97% of ALS patient brains (Bodansky et al, 2010; Ling et al, 2010; Tan et al, 2017), where it forms pathological inclusions of phosphorylated and ubiquitinated proteins in the cytoplasm of motor neurons (Neumann et al, 2006; Hasegawa et al, 2008; Igaz et al, 2008; Kametani et al, 2016; Buratti, 2018). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.