In ALS the majority of aggregates are ubiquitinated inclusions (Wood et al., 2003) typically containing trans-activation response element (TAR) DNA binding protein 43 (TDP-43; Neumann et al., 2006) and also mutant Cu, Zn-superoxide dismutase (SOD1) in SOD1-linked familial ALS (Shibata et al., 1996) and mutant SOD1 mouse (Watanabe et al., 2001) and cell culture models (Lee et al., 2002). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.