Furthermore, reduced expression of UPS components in the spinal cord of SOD1G93A transgenic mice has been reported (Basso et al., 2009; Marino et al., 2015), and ALS mutant SOD1 itself is poly-ubiquitinated and cleared by the proteasome (Niwa et al., 2002; Urushitani et al., 2004). The gene discussed is SOD1; the disease is amyotrophic lateral sclerosis.