{"method": "X-RAY DIFFRACTION", "mutation_s_field": "S134N ", "ec_number": "1.15.1.1", "description": "S134N 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. There is a mixture of copper and zinc ions in the Cu-binding site and zinc alone is bound in the Zn-binding site in the subunits of S134N SOD1.", "ligstr": "CSX:A:C3 H7 N O3 S:137.16:S-OXY CYSTEINE:C([C@@H](C(=O)O)N)[S@H]=O:InChI=1S/C3H7NO3S/c4-2(1-8-7)3(5)6/h2,8H,1,4H2,(H,5,6)/t2-/m0/s1:BHLMCOCHAVMHLD-REOHCLBHSA-N;SO4:A:O4 S -2:96.06:SULFATE ION:[O-]S(=O)(=O)[O-]:InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2:QAOWNCQODCNURD-UHFFFAOYSA-L;ZN:A:Zn 2:65.41:ZINC ION:[Zn+2]:InChI=1S/Zn/q+2:PTFCDOFLOPIGGS-UHFFFAOYSA-N;CSX:B:C3 H7 N O3 S:137.16:S-OXY CYSTEINE:C([C@@H](C(=O)O)N)[S@H]=O:InChI=1S/C3H7NO3S/c4-2(1-8-7)3(5)6/h2,8H,1,4H2,(H,5,6)/t2-/m0/s1:BHLMCOCHAVMHLD-REOHCLBHSA-N;SO4:B:O4 S -2:96.06:SULFATE ION:[O-]S(=O)(=O)[O-]:InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2:QAOWNCQODCNURD-UHFFFAOYSA-L;ZN:B:Zn 2:65.41:ZINC ION:[Zn+2]:InChI=1S/Zn/q+2:PTFCDOFLOPIGGS-UHFFFAOYSA-N", "pmid": "12754496", "remarks": "Crystal Structure of Familial ALS Mutant S134N of human Cu,Zn Superoxide Dismutase (CuZnSOD)", "amyloid_non_amyloid": "Amyloid", "type": "Protein", "inchi": "InChI=1S/C3H7NO3S/c4-2(1-8-7)3(5)6/h2,8H,1,4H2,(H,5,6)/t2-/m0/s1%%InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2%%InChI=1S/Zn/q+2%%InChI=1S/C3H7NO3S/c4-2(1-8-7)3(5)6/h2,8H,1,4H2,(H,5,6)/t2-/m0/s1%%InChI=1S/H2O4S/c1-5(2,3)4/h(H2,1,2,3,4)/p-2%%InChI=1S/Zn/q+2", "resolution": "1.3", "species": "Homo sapiens", "secondary_structure": "ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEENTKTGNAGSRLACGVIGIAQ#CEEEEEEEBCSSSCEEEEEEEESSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGSCSBCCTTCCCCCCTTCSSCCTTEEEEEEECTTSCEEEEEEESSSBSSSTTBCTTSEEEEESSCCCTTCCCCCCCCCSCSCCSEEEEEECEECC&ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEENTKTGNAGSRLACGVIGIAQ#CCEEEEEEBCSSSCEEEEEEECCSTTSCEEEEEEEESCCSEEEEEEEESCCCCTTGGGGSCSBCCCCCCCCCCCCCCCCCTTEEEEEEECTTSCEEEEEEESSCBSSSTTBCTTSEEEEESSCCCCCCCCCCCCCCCCCCCSEEEEEECEECC", "chain_id": "A%%A%%A%%B%%B%%B", "gene_names": "SOD1", "r_value_free": "0.193", "pdb_id": "1OZU", "reference": "Nat Struct Biol. 2003 Jun;10(6):461-7", "keyword": "Superoxide dismutase [Cu-Zn]", "protein_name": "Superoxide dismutase [Cu-Zn]", "ligand_formula": "C3 H7 N O3 S%%O4 S -2%%Zn 2%%C3 H7 N O3 S%%O4 S -2%%Zn 2", "peptide_protein_sequence": "chain-ID A: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEENTKTGNAGSRLACGVIGIAQ; chain-ID B: ATKAVCVLKGDGPVQGIINFEQKESNGPVKVWGSIKGLTEGLHGFHVHEFGDNTAGCTSAGPHFNPLSRKHGGPKDEERHVGDLGNVTADKDGVADVSIEDSVISLSGDHCIIGRTLVVHEKADDLGKGGNEENTKTGNAGSRLACGVIGIAQ", "entry": "S-0050", "length": "153", "uniprot_ac": "P00441", "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.", "ligand_smiles": "C([C@@H](C(=O)O)N)[S@H]=O%%[O-]S(=O)(=O)[O-]%%[Zn+2]%%C([C@@H](C(=O)O)N)[S@H]=O%%[O-]S(=O)(=O)[O-]%%[Zn+2]", "ligand_mw": "137.16%%96.06%%65.41%%137.16%%96.06%%65.41", "alternative_name": "Superoxide dismutase 1", "ligand_name": "S-OXY CYSTEINE%%SULFATE ION%%ZINC ION%%S-OXY CYSTEINE%%SULFATE ION%%ZINC ION", "pdb_classification": "OXIDOREDUCTASE", "global_stoichiometry": "Homo 2-mer - A2 ", "inchi_key": "BHLMCOCHAVMHLD-REOHCLBHSA-N%%QAOWNCQODCNURD-UHFFFAOYSA-L%%PTFCDOFLOPIGGS-UHFFFAOYSA-N%%BHLMCOCHAVMHLD-REOHCLBHSA-N%%QAOWNCQODCNURD-UHFFFAOYSA-L%%PTFCDOFLOPIGGS-UHFFFAOYSA-N", "ligand_id": "CSX%%SO4%%ZN%%CSX%%SO4%%ZN"}