SOD1:Zn2+ apoenzyme binds CCS:Cu1+ (mitochondrial)

Stable Identifier
Reaction [binding]
Homo sapiens
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As inferred from the cytosolic reaction and from the mouse mitochondrial reaction, Copper chaperone of superoxide dismutase (CCS) transfers a copper(I) atom to a SOD1 monomer that already contains a Zn atom. The reaction proceeds by a two step mechanism in which SOD1 first forms heterodimers with CCS. The amounts of CCS and SOD1 in the intermembrane space appear to be regulated by the concentration of oxygen. Mutations in SOD1 are responsible for familial amyotrophic lateral sclerosis (fALS) and cause unregulated localization and aggregation of SOD1 in the intermembrane space (reviewed in Kawamata and Manfredi 2010).

Literature References
PubMed ID Title Journal Year
20367259 Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space

Kawamata, H, Manfredi, G

Antioxid. Redox Signal. 2010
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