HCCS and amyotrophic lateral sclerosis: 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.