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Decreased copper incorporation into SOD1 is detected in fibroblasts derived from CCS−/− mice. Primary fibroblast cultures were isolated from wild-type, heterozygous, and homozygous CCS knockout mice. (a) Protein lysates (50 μg) immunoblotted with antisera to SOD1. (b) SOD1 gel activity assay of 50 μg protein extract. (c) Protein lysate (50 μg) of 64Cu-labeled fibroblasts.
