2rli
From Proteopedia
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==Overview== | ==Overview== | ||
| - | Human Sco2 is a mitochondrial membrane-bound protein involved in copper | + | 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. |
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| + | ==Disease== | ||
| + | Known disease associated with this structure: Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604272 604272]] | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | A | + | 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:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=17850752 17850752] |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Single protein]] | [[Category: Single protein]] | ||
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[[Category: Bertini, I.]] | [[Category: Bertini, I.]] | ||
[[Category: Ciofi-baffoni, S.]] | [[Category: Ciofi-baffoni, S.]] | ||
| - | [[Category: Gerothanassis, I | + | [[Category: Gerothanassis, I P.]] |
[[Category: Leontari, I.]] | [[Category: Leontari, I.]] | ||
[[Category: Martinelli, M.]] | [[Category: Martinelli, M.]] | ||
| - | [[Category: SPINE, Structural | + | [[Category: SPINE, Structural Proteomics in Europe.]] |
[[Category: Wang, S.]] | [[Category: Wang, S.]] | ||
[[Category: CU1]] | [[Category: CU1]] | ||
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[[Category: thioredoxin fold]] | [[Category: thioredoxin fold]] | ||
| - | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on | + | ''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 18:48:21 2008'' |
Revision as of 16:48, 21 February 2008
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Solution structure of Cu(I) human Sco2
Contents |
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
Categories: Homo sapiens | Single protein | Banci, L. | Bertini, I. | Ciofi-baffoni, S. | Gerothanassis, I P. | Leontari, I. | Martinelli, M. | SPINE, Structural Proteomics in Europe. | Wang, S. | CU1 | Copper protein | Metal transport | Spine | Spine2-complexes | Structural genomics | Structural proteomics in europe | Thioredoxin fold
