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). This evidence concerns the gene SOD1 and amyotrophic lateral sclerosis.