2rli

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Solution structure of Cu(I) human Sco2

Contents 1 Overview 2 Disease 3 About this Structure 4 Reference

Overview

Human Sco2 is a mitochondrial membrane-bound protein involved in copper supply for the assembly of cytochrome c oxidase in eukaryotes. Its precise action is not yet understood. We report here a structural and dynamic characterization by NMR of the apo and copper(I) forms of the soluble fragment. The structural and metal binding features of human Cu(I)Sco2 are similar to the more often studied Sco1 homolog, although the dynamic properties and the conformational disorder are quite different when the apo forms and the copper(I)-loaded forms of the two proteins are compared separately. Such differences are accounted for in terms of the different physicochemical properties in strategic protein locations. The misfunction of the known pathogenic mutations is discussed on the basis of the obtained structure.

Disease

Known disease associated with this structure: Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency OMIM:[604272]

About this Structure

2RLI is a Single protein structure of sequence from Homo sapiens with as ligand. Full crystallographic information is available from OCA.

Reference

A structural characterization of human SCO2., Banci L, Bertini I, Ciofi-Baffoni S, Gerothanassis IP, Leontari I, Martinelli M, Wang S, Structure. 2007 Sep;15(9):1132-40. PMID:17850752

Page seeded by OCA on Thu Feb 21 18:48:21 2008