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

@article{Leary2009HumanSI,
  title={Human SCO2 is required for the synthesis of CO II and as a thiol-disulphide oxidoreductase for SCO1.},
  author={Scot C Leary and Florin Sasarman and Tamiko Nishimura and Eric A Shoubridge},
  journal={Human molecular genetics},
  year={2009},
  volume={18 12},
  pages={2230-40}
}
Human SCO1 and SCO2 code for essential metallochaperones with ill-defined functions in the biogenesis of the CuA site of cytochrome c oxidase subunit II (CO II). Here, we have used patient cell lines to investigate the specific roles of each SCO protein in this pathway. By pulse-labeling mitochondrial translation products, we demonstrate that the synthesis of CO II is reduced in SCO2, but not in SCO1, cells. Despite this biosynthetic defect, newly synthesized CO II is more stable in SCO2 cells… CONTINUE READING
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