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Article

The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis.

Cell metabolism - 1 Jan 2007

Leary Scot C, Cobine Paul A, Kaufman Brett A, Guercin Guy-Hellen, Mattman Andre, Palaty Jan, Lockitch Gillian, Winge Dennis R, Rustin Pierre, Horvath Rita, Shoubridge Eric A

Abstract excerpt

Human SCO1 and SCO2 are metallochaperones that are essential for the assembly of the catalytic core of cytochrome c oxidase (COX). Here we show that they have additional, unexpected roles in cellular copper homeostasis. Mutations in either SCO result in a cellular copper deficiency that is both tissue and allele specific. This phenotype can be dissociated from the defects in COX assembly and is suppressed by...

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Source

Identifiers

PMID: 17189203
DOI: 10.1016/j.cmet.2006.12.001

Genes

SCO1
SCO2

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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The human cytochrome c oxidase assembly factors SCO1 and SCO2 have regulatory roles in the maintenance of cellular copper homeostasis.PMID 17189203Read ↗

Human SCO1 and SCO2 have independent, cooperative functions in copper delivery to cytochrome c oxidase.Semantic proximity Read ↗

Crystal structure of human SCO1: implications for redox signaling by a mitochondrial cytochrome c oxidase "assembly" protein.Semantic proximity Read ↗

Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1.Semantic proximity Read ↗

Heart is the most susceptible organ in an isogenic background to loss of function mutations in the mitochondrial metallochaperone SCO1.Semantic proximity Read ↗

Cytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts.Semantic proximity Read ↗

COA6 Facilitates Cytochrome c Oxidase Biogenesis as Thiol-reductase for Copper Metallochaperones in Mitochondria.Semantic proximity Read ↗

Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1.Semantic proximity Read ↗

The P174L mutation in human Sco1 severely compromises Cox17-dependent metallation but does not impair copper binding.Semantic proximity Read ↗

A human SCO2 mutation helps define the role of Sco1p in the cytochrome oxidase assembly pathway.Semantic proximity Read ↗

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Novel mutations in SCO1 as a cause of fatal infantile encephalopathy and lactic acidosis.Semantic proximity Read ↗

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