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116,126 Specifically, CCS-overexpressing SOD G93A mice exhibit significantly decreased COX activity when these mice reached the end stage of disease, whereas SOD G93A mice not overexpressing CCS had no apparent decrease in COX activity at the same stage, 126 supporting the hypothesis that overexpressing CCS increases the delivery of Cu to SOD1 while reducing the delivery of Cu to other enzymes (e.g., mitochondrial cytochrome c oxidase), thus leading to additional toxic effects