SUBCELLULAR LOCALIZATION
| intracellular
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| intracellular,cytoplasm,organelle,mitochondria,interspace
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| intracellular,cytoplasm,cytosolic
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| intracellular,nucleus
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text
| colocalyzing with SOD1 |
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CCS localization in the cytosol prevents SOD1 mitochondrial import, whereas CCS in mitochondria increases it |
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mitochondrial distribution is regulated by the CHCHD40 disulfide relay system in a redox-dependent manner |
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present in the cytosol and in the intermembrane space of mitochondria |
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CCS import depends on the presence of mature CCS in the mitochondria |
basic FUNCTION
| mediating copper incorporation in superoxide dismutase |
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may activate copper/zinc superoxide dismutase through direct insertion of the Cu cofactor |
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may be playing a role in prioritizing the utilization of copper when it is scarce |
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likely exhibits disulfide isomerase activity |
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involved in one activation pathway of SOD1 (CCS-dependent pathway) |
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necessary for expression of an active, copper-bound form of superoxide dismutase (SOD1) in spite of the high affinity of SOD1 for copper and the high intracellular concentrations of both SOD1 and copper |
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metallochaperone protein activating the target enzyme through direct insertion of the copper cofactor and apparently functions to protect the metal ion from binding to intracellular copper scavengers |
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mediator of copper delivery to XIAP in cells |
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promotes SOD1 maturation and retention in the intermembrane space |
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specifically delivers copper (Cu) to copper, zinc superoxide dismutase (SOD1) in cytoplasm of mammalian cells |
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the amount of CCS protein exceeds that required to supply Cu to SOD1 in the cells |
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CCS is the physiological partner for the complex mechanism of SOD1 maturation |
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facilitates copper trafficking to the mitochondria, but does not affect the transfer of copper to the cytochrome c oxidase (CCO) |
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serves as a specialized import receptor in mitochondria that facilitates the import and folding of SOD1 and CCS, thereby extending the substrate spectrum of oxidation-dependent protein import in the mitochondrial intermembrane space |