CCS transfers Cu to SOD1 and oxidizes cysteine residues in SOD1

Stable Identifier
R-HSA-3299753
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
Summation

Copper chaperone of superoxide dismutase (CCS) transfers a copper(I) atom to a SOD1 monomer that already contains a Zn atom. After initial heterodimerization between SOD1 and CCS, the copper atom is transferred, intramolecular cysteine disulfide bonds are formed in SOD1, and SOD1 dimerizes (Banci et al. 2012, Casareno et al. 1998, Culotta et al. 1997, Rae et al. 2001, Brown et al. 2004, Carroll et al. 2006, Kawamata and Manfredi 2008). The transfer of copper to SOD1 requires oxygen but it is unknown at which step the oxygen acts (Brown et al. 2004). There is also a CCS-independent, oxygen-independent pathway of maturation of SOD1 (Leitch et al. 2009) whose molecular details and physiological role are not well characterized.

Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
protein disulfide oxidoreductase activity of CCS:Zn2+:2xCu1+:SOD1:Zn2+ [cytosol]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed