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

@article{Stiburek2005TissuespecificCC,
  title={Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1.},
  author={Lukas Stiburek and Katerina Vesela and Hana Hansikova and Petr Pecina and Marketa Tesarova and Leona Cern{\'a} and Josef Hou{\vs}těk and Jiř{\'i} Zeman},
  journal={The Biochemical journal},
  year={2005},
  volume={392 Pt 3},
  pages={625-32}
}
The biogenesis of eukaryotic COX (cytochrome c oxidase) requires several accessory proteins in addition to structural subunits and prosthetic groups. We have analysed the assembly state of COX and SCO2 protein levels in various tissues of six patients with mutations in SCO2 and SURF1. SCO2 is a copper-binding protein presumably involved in formation of the Cu(A) centre of the COX2 subunit. The function of SURF1 is unknown. Immunoblot analysis of native gels demonstrated that COX holoenzyme is… CONTINUE READING

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