{"reference": "Nat Struct Biol. 2003 Jun;10(6):461-7", "alternative_name": "Superoxide dismutase 1", "entry": "S-0049", "gene_names": "SOD1", "remarks": "Crystal Structure of apo-H46R Familial ALS Mutant human Cu,Zn Superoxide Dismutase (CuZnSOD); linear amyloid-like filaments ", "secondary_structure": "ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCCCCCCCCCCCCCCCCCCEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCTTCSSCCCCCCCCCCCCEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCTTCSSCCCCCCCCCCCCEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCSCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCCSCSCCCCCCCCCCCCCEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCCCCCCCCCCCCCCCCCCEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCCCCCCCCCCCCCCCCSCEEEEEEEEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCTTCCCCCCTTCSSSCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCTTCSSSHHHHHHTTCCSEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGGCSBCCTTCCCCCCSSSSSCCTTEEEEEEECTTSCEEEEEEESSSBSSGGGBCTTSEEEEESSCCCTTCSSSTTHHHHTTCCSEEEEEECEECC", "chain_id": "", "inchi": "", "amyloid_non_amyloid": "Amyloid", "pmid": "12754496", "ligand_smiles": "", "keyword": "Superoxide dismutase [Cu-Zn]", "peptide_protein_sequence": "chain-ID G: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID H: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID I: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID J: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID K: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID L: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID M: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ; chain-ID N: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFRVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEESTKTGNAGSRLACGVIGIAQ", "uniprot_ac": "P00441", "protein_name": "Superoxide dismutase [Cu-Zn]", "r_value_free": "0.27", "mutation_s_field": "apo-H46R", "ligand_mw": "", "pdb_id": "1OZT", "inchi_key": "", "ec_number": "1.15.1.1", "pdb_classification": "OXIDOREDUCTASE", "type": "Protein", "author": "Elam, J.S., Taylor, A.B., Strange, R., Antonyuk, S., Doucette, P.A., Rodriguez, J.A., Hasnain, S.S., Hayward, L.J., Valentine, J.S., Yeates, T.O., Hart, P.J.", "species": "Homo sapiens", "ligand_id": "", "method": "X-RAY DIFFRACTION", "ligand_name": "", "global_stoichiometry": "Homo 2-mer - A2 ", "resolution": "2.5", "ligand_formula": "", "length": "153", "ligstr": "", "description": "H46R SOD1 structure is preserve relative to the wild-type protein, it exhibit significant disorder and conformational change in its zinc and electrostatic loop elements. Amyloid-like filaments of pathogenic SOD1 dimers are linear. Residues 125\u00e2\u20ac\u201c131 of the electrostatic loop adopt an extended, non-native conformation and participate in extensive hydrogen bonding and apolar interactions with the deprotected surface depression on the Beta-barrel of a neighboring SOD1 dimer. apo-H46R subunits are completely devoid of both copper and zinc ions in their binding sites."}