1AZV

FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)

1AZV の概要

• エントリーDOI: 10.2210/pdb1azv/pdb
• 分子名称: COPPER/ZINC SUPEROXIDE DISMUTASE, COPPER (II) ION, ZINC ION, ... (4 entities in total)
• 機能のキーワード: oxidoreductase, superoxide acceptor, familial amyotrophic lateral sclerosis mutant
• 由来する生物種: Homo sapiens (human)
• 細胞内の位置: Cytoplasm: P00441
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 32113.32

構造登録者

Hart, P.J.,Liu, H.,Pellegrini, M.,Nersissian, A.M.,Gralla, E.B.,Valentine, J.S.,Eisenberg, D. (登録日: 1997-11-21, 公開日: 1998-02-25, 最終更新日: 2024-10-30)

主引用文献

Hart, P.J.,Liu, H.,Pellegrini, M.,Nersissian, A.M.,Gralla, E.B.,Valentine, J.S.,Eisenberg, D.
Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis.
Protein Sci., 7:545-555, 1998

PubMed Abstract: The X-ray crystal structure of a human copper/zinc superoxide dismutase mutant (G37R CuZnSOD) found in some patients with the inherited form of Lou Gehrig's disease (FALS) has been determined to 1.9 angstroms resolution. The two SOD subunits have distinct environments in the crystal and are different in structure at their copper binding sites. One subunit (subunit[intact]) shows a four-coordinate ligand geometry of the copper ion, whereas the other subunit (subunit[broken]) shows a three-coordinate geometry of the copper ion. Also, subunit(intact) displays higher atomic displacement parameters for backbone atoms ((B) = 30 +/- 10 angstroms2) than subunit(broken) ((B) = 24 +/- 11 angstroms2). This structure is the first CuZnSOD to show large differences between the two subunits. Factors that may contribute to these differences are discussed and a possible link of a looser structure to FALS is suggested.

PubMed: 9541385

実験手法

X-RAY DIFFRACTION (1.9 Å)