Copper incorporation into SOD1 is impaired in CCS
knockout mice. Protein extracts from various tissues of wild-type,
heterozygous, and homozygous CCS knockout mice and
homozygous SOD1 knockout mice were obtained. (a)
SDS/PAGE gel of liver (50 μg), kidney (75 μg), brain and spinal
cord (100 μg), immunoblotted with antisera to SOD1.
(b) SOD1 gel activity assay on same samples as
a. (c) 64Cu incorporation
into SOD1, 100 μg of protein lysate for each tissue.
(d) Analysis of serum samples from wild-type,
heterozygous, and homozygous CCS knockout mice and
homozygous ceruloplasmin knockout mice. (Top to
Bottom) Immunoblot with antisera to human ceruloplasmin,
64Cu incorporation to ceruloplasmin, and ceruloplasmin
activity gel assay.