Several other lines of evidence also indicate that the hCCS-SOD1 interaction is effected by disease causing mutations: 1) hCCS overexpression in a G93A SOD1 mouse causes extreme ALS-like symptoms and drastically reduces life-expectancy43, 2) hCCS over-expression increases the proportion of disulphide oxidized wild-type SOD1 but not ALS mutant SOD144, 3) SOD1 is found to be metal depleted and disulphide reduced in the inclusions found in mutant SOD1 transgenic animal models11, 12, 13. Here, SOD1 is linked to amyotrophic lateral sclerosis.