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CCS

Function

Delivers copper to copper zinc superoxide dismutase (SOD1).

Post-translational modifications

Ubiquitinion by XIAP/BIRC4 leads to enhancement of its chaperone activity toward its physiologic target, SOD1, rather than proteasomal degradation. XIAP/BIRC4 preferentially ubiquitinates at Lys-241.

Sequence Similarities

In the C-terminal section; belongs to the Cu-Zn superoxide dismutase family.

Tissue Specificity

Ubiquitous.

Cellular localization

Alternative names

Copper chaperone for superoxide dismutase, Superoxide dismutase copper chaperone, CCS

swissprot:O14618 omim:603864 entrezGene:9973