Title : COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux.

Pub. Date : 2013 Mar

PMID : 23345593






11 Functional Relationships(s)
Download
Sentence
Compound Name
Protein Name
Organism
1 COX19 mediates the transduction of a mitochondrial redox signal from SCO1 that regulates ATP7A-mediated cellular copper efflux. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
2 SCO1 and SCO2 are metallochaperones whose principal function is to add two copper ions to the catalytic core of cytochrome c oxidase (COX). Copper synthesis of cytochrome C oxidase 1 Homo sapiens
3 However, affected tissues of SCO1 and SCO2 patients exhibit a combined deficiency in COX activity and total copper content, suggesting additional roles for these proteins in the regulation of cellular copper homeostasis. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
4 However, affected tissues of SCO1 and SCO2 patients exhibit a combined deficiency in COX activity and total copper content, suggesting additional roles for these proteins in the regulation of cellular copper homeostasis. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
5 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
6 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
7 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
8 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
9 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
10 Here we show that both the redox state of the copper-binding cysteines of SCO1 and the abundance of SCO2 correlate with cellular copper content and that these relationships are perturbed by mutations in SCO1 or SCO2, producing a state of apparent copper overload. Copper synthesis of cytochrome C oxidase 1 Homo sapiens
11 These results demonstrate that COX19 is necessary for the transduction of a SCO1-dependent mitochondrial redox signal that regulates ATP7A-mediated cellular copper efflux. Copper synthesis of cytochrome C oxidase 1 Homo sapiens