CCS

CCS (Copper Chaperone for Superoxide Dismutase), also known as SOD1 copper chaperone or LYS7 ortholog, is a conserved metallochaperone. It binds cytosolic Cu(I) via its N-terminal domain, interacts with apo-SOD1, and inserts copper cofactor to activate SOD1, regulating antioxidant defense and copper homeostasis linked to NFE2L2 and HIF-1 pathways.
CCS dysfunction correlates with amyotrophic lateral sclerosis (ALS), Wilson disease, and Mednik syndrome, driven by impaired SOD1 maturation and copper dysregulation. Targeting CCS-SOD1 interaction with small molecules or copper modulators is under preclinical investigation for ALS and oxidative stress-related disorders.