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Article

Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space.

Antioxidants & redox signaling - 1 Nov 2010

Kawamata Hibiki, Manfredi Giovanni

Abstract excerpt

Cu, Zn, superoxide dismutase (SOD1) is a ubiquitous enzyme localized in multiple cellular compartments, including mitochondria, where it concentrates in the intermembrane space (IMS). Similar to other small IMS proteins, the import and retention of SOD1 in the IMS is linked to its folding and maturation, involving the formation of critical intra- and intermolecular disulfide bonds. Therefore, the cysteine...

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Identifiers

PMID: 20367259
DOI: 10.1089/ars.2010.3212
PMCID: PMC2962758

Genes

SOD1
CCS

Provenance

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