It has been estimated that independent copper binding still represents a substantial concentration of intracellular Cu2+ (10 μM) in SOD1-overexpressing transgenic mice [69], suggesting a possibility of 10 μM Cu2+ to induce the oxidation and aggregation of ALS-associated SOD1 mutants in vivo even in the absence of CCS. This evidence concerns the gene CCS and amyotrophic lateral sclerosis.