Following cleavage, the cytosolic domain of ATF6 is translocated to nucleus where it activates UPR-related genes (Gotoh et al., 2002; Yoshida et al., 2003) Elevated levels of ATF6 have been reported in ALS patients and G93A-SOD1 mice (Atkin et al., 2006, 2008). The gene discussed is ATF6; the disease is amyotrophic lateral sclerosis.