Article
Human recombinant mutated forms of the mitochondrial COX assembly Sco2 protein differ from wild-type in physical state and copper binding capacity.
Molecular genetics and metabolism - 1 Mar 2004
Foltopoulou Parthena F, Zachariadis George A, Politou Anastasia S, Tsiftsoglou Asterios S, Papadopoulou Lefkothea C
Abstract excerpt
The human Sco2 protein is a cytochrome c oxidase assembly protein that participates in mitochondrial copper pathway, acting downstream of Cox17 protein. In a previous work, we detected mutations in the human SCO2 gene in three unrelated infants with fatal cardioencephalomyopathy and COX deficiency. In this study, full-length processed recombinant wild-type and two mutated forms of hSco2p (w/t-rhSco2p,...
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