PMID:15659396
| Citation |
Williams, JC, Sue, C, Banting, GS, Yang, H, Glerum, DM, Hendrickson, WA and Schon, EA (2005) Crystal structure of human SCO1: implications for redox signaling by a mitochondrial cytochrome c oxidase "assembly" protein. J. Biol. Chem. 280:15202-11 |
|---|---|
| Abstract |
Human SCO1 and SCO2 are copper-binding proteins involved in the assembly of mitochondrial cytochrome c oxidase (COX). We have determined the crystal structure of the conserved, intermembrane space core portion of apo-hSCO1 to 2.8 A. It is similar to redox active proteins, including thioredoxins (Trx) and peroxiredoxins (Prx), with putative copper-binding ligands located at the same positions as the conserved catalytic residues in Trx and Prx. SCO1 does not have disulfide isomerization or peroxidase activity, but both hSCO1 and a sco1 null in yeast show extreme sensitivity to hydrogen peroxide. Of the six missense mutations in SCO1 and SCO2 associated with fatal mitochondrial disorders, one lies in a highly conserved exposed surface away from the copper-binding region, suggesting that this region is involved in protein-protein interactions. These data suggests that SCO functions not as a COX copper chaperone, but rather as a mitochondrial redox signaling molecule. |
| Links |
PubMed Online version:10.1074/jbc.M410705200 |
| Keywords |
Amino Acid Sequence; Animals; Binding Sites; Cattle; Chromatography; Copper/chemistry; Crystallography, X-Ray; Cysteine/chemistry; Disulfides/chemistry; Electron Transport Complex IV/chemistry; Genotype; Humans; Hydrogen Peroxide/chemistry; Hydrogen Peroxide/pharmacology; Ligands; Membrane Proteins/chemistry; Mitochondria/metabolism; Mitochondrial Proteins/chemistry; Models, Molecular; Molecular Chaperones/chemistry; Molecular Sequence Data; Mutation; Mutation, Missense; Oxidation-Reduction; Peroxidases/chemistry; Peroxides/chemistry; Point Mutation; Protein Conformation; Protein Structure, Tertiary; Sequence Homology, Amino Acid; Thioredoxins/chemistry; Time Factors |
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