Article
Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1.
The Biochemical journal - 15 Dec 2005
Stiburek Lukas, Vesela Katerina, Hansikova Hana, Pecina Petr, Tesarova Marketa, Cerna Leona, Houstek Josef, Zeman Jiri
Abstract excerpt
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...
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