Diminished SOD1 enzymatic activity in CCS knockout
mice. (a) SOD1 activity assay gel of 25 or 50 μg
protein extracts, respectively, from brain, spinal cord, muscle, and
liver or from lung, heart, and kidney of wild-type (lanes 2, 5, 8, 11,
14, 17, and 20), heterozygous (lanes 3, 6, 9, 12, 15, 18, and 21), and
homozygous (lanes 4, 7, 10, 13, 16, 19, and 22) CCS
knockout mice. Arrow denote the position of mouse SOD1. Purified human
erythrocyte SOD1 is shown in lane 1. (b) SOD1 activity
determined from tissue extracts indicated by using a cytochrome
c/xanthine oxidase method. The averages of SOD1
activities +/− standard deviations from three mice for each CCS
genotype are shown. “Wild-type” values, as previously reported
(21), are included for comparison.