1AZV

FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)

Summary for 1AZV

• Entry DOI: 10.2210/pdb1azv/pdb
• Descriptor: COPPER/ZINC SUPEROXIDE DISMUTASE, COPPER (II) ION, ZINC ION, ... (4 entities in total)
• Functional Keywords: oxidoreductase, superoxide acceptor, familial amyotrophic lateral sclerosis mutant
• Biological source: Homo sapiens (human)
• Cellular location: Cytoplasm: P00441
• Total number of polymer chains: 2
• Total formula weight: 32113.32

Authors

Hart, P.J.,Liu, H.,Pellegrini, M.,Nersissian, A.M.,Gralla, E.B.,Valentine, J.S.,Eisenberg, D. (deposition date: 1997-11-21, release date: 1998-02-25, Last modification date: 2024-10-30)

Primary citation

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

Experimental method

X-RAY DIFFRACTION (1.9 Å)