SOD1 and amyotrophic lateral sclerosis: Notably, however, SOD1 aggregates are a key ALS hallmark but are absent in spinal cords from CCS/G93-SOD1 mice [149], and mortality in these mice may be explained entirely by copper deficiency during the first 10 days of life [150], in some ways resembling Menke’s disease better than adult-onset ALS [151].