TARDBP and amyotrophic lateral sclerosis: Both familial ALS caused by TARDBP mutations and sporadic ALS have distinguishing features of clinical pathology in the affected motor neurons, which include the loss of TDP-43 from the nucleus and abnormal formation of cytoplasmic aggregations containing hyper-phosphorylated and ubiquitinated TDP-43 (Arai et al, 2006; Chen-Plotkin et al, 2010; Neumann et al, 2006).