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Article

Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations.

The Biochemical journal - 15 Apr 2002

Salviati Leonardo, Hernandez-Rosa Evelyn, Walker Winsome F, Sacconi Sabrina, DiMauro Salvatore, Schon Eric A, Davidson Mercy M

Abstract excerpt

Human SCO2 is a nuclear-encoded Cu-binding protein, presumed to be responsible for the insertion of Cu into the mitochondrial cytochrome c oxidase (COX) holoenzyme. Mutations in SCO2 are associated with cardioencephalomyopathy and COX deficiency. Studies in yeast and bacteria have shown that Cu supplementation can restore COX activity in cells harbouring mutations in genes involving Cu transport. Therefore we...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 11931660
DOI: 10.1042/0264-6021:3630321
PMCID: PMC1222481

Genes

SCO2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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Copper supplementation restores cytochrome c oxidase activity in cultured cells from patients with SCO2 mutations.PMID 11931660Read ↗

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Human recombinant mutated forms of the mitochondrial COX assembly Sco2 protein differ from wild-type in physical state and copper binding capacity.Semantic proximity Read ↗

Mutations in SCO2 are associated with a distinct form of hypertrophic cardiomyopathy and cytochrome c oxidase deficiency.Semantic proximity Read ↗

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