Other postulated mechanisms of neuronal death in ALS include glutamate excitotoxicity (Rothstein, 2009), excessive generation of reactive oxygen and nitrogen species (Barber and Shaw, 2010), mitochondrial dysfunction (Rothstein, 2009), induction of endoplasmic reticulum (ER) stress (Atkin et al., 2008), axonal deterioration, and deposition of toxic ubiquitinated neuronal inclusions, where transactive response DNA binding protein 43 kDa (TDP-43) and fused in sarcoma (FUS) are major protein components (Arai et al., 2006; Rothstein, 2009). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.