SOD1 and amyotrophic lateral sclerosis: One of the principal causes of FALS is the mutation in the SOD1 gene.114 In the context of ALS, the interaction of CCS with mutant SOD1 is faulty, resulting in decreased delivery of Cu to the mitochondria and the accumulation of the less-stabilized SOD1, which is prone to acquire pro-oxidants, consequently causing a toxic gain-of-function effect in motor neurons.115 Interestingly, a previous study has shown that overexpressing CCS accelerates neurological deficits and shortens the life span of mice carrying the G93A mutation in SOD1 (SOD1G93A mice).116