2af2 | pdb_00002af2
Solution structure of disulfide reduced and copper depleted Human Superoxide Dismutase
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| 2af2 | |||||||||||||
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| Ligands: | ZN | ||||||||||||
| Gene: | SOD1 (Homo sapiens) | ||||||||||||
| Activity: | Superoxide dismutase, with EC number 1.15.1.1 | ||||||||||||
| Coordinates: | save as pdb, mmCIF, xml | ||||||||||||
Overview
SOD1 has to undergo several post-translational modifications before reaching its mature form. The protein requires insertion of zinc and copper atoms, followed by the formation of a conserved S-S bond between Cys-57 and Cys-146 (human numbering), which makes the protein fully active. In this report an NMR structural investigation of the reduced SH-SH form of thermostable E,Zn-as-SOD1 (E is empty; as is C6A, C111S) is reported, characterizing the protein just before the last step leading to the mature form. The structure is compared with that of the oxidized S-S form as well as with that of the yeast SOD1 complexed with its copper chaperone, CCS. Local conformational rearrangements upon disulfide bridge reduction are localized in the region near Cys-57 that is completely exposed to the solvent in the present structure, at variance with the oxidized forms. There is a local disorder around Cys-57 that may serve for protein-protein recognition and may possibly be involved in intermolecular S-S bonds in familial amyotrophic lateral sclerosis-related SOD1 mutants. The structure allows us to further discuss the copper loading mechanism in SOD1.
Disease
Known disease associated with this structure: Amyotrophic lateral sclerosis, due to SOD1 deficiency OMIM:[147450]
About this Structure
2AF2 is a Single protein structure of sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
Human SOD1 before harboring the catalytic metal: solution structure of copper-depleted, disulfide-reduced form., Banci L, Bertini I, Cantini F, D'Amelio N, Gaggelli E, J Biol Chem. 2006 Jan 27;281(4):2333-7. Epub 2005 Nov 14. PMID:16291742
Page seeded by OCA on Thu Mar 20 15:48:52 2008
Proteopedia Page Contributors and Editors (what is this?)
- Pages with broken file links
- Homo sapiens
- Single protein
- Superoxide dismutase
- Amelio, N D.
- Banci, L.
- Bertini, I.
- Cantini, F.
- Gaggelli, E.
- SPINE, Structural Proteomics in Europe.
- ZN
- Copper depleted protein
- Disulfide bond reduced
- Homodimeric protein
- Human superoxide dismutase
- Nmr
- Solution structure
- Spine
- Structural genomic
- Structural proteomics in europe
