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

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 supp...

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Main Authors: Salviati, Leonardo, Hernandez-Rosa, Evelyn, Walker, Winsome F, Sacconi, Sabrina, DiMauro, Salvatore, Schon, Eric A, Davidson, Mercy M
Format: Artigo
Sprog:Inglês
Udgivet: 2002
Fag:
Research Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC1222481/
https://ncbi.nlm.nih.gov/pubmed/11931660
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