› 1azv

X-ray diffraction
1.9Å resolution

FAMILIAL ALS MUTANT G37R CUZNSOD (HUMAN)

Released:
DOI: 10.2210/pdb1azv/pdb
PDB entry 1azv coloured by chain, front view.
PDB entry 1azv coloured by chain, side view.
PDB entry 1azv coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Subunit asymmetry in the three-dimensional structure of a human CuZnSOD mutant found in familial amyotrophic lateral sclerosis.
Hart PJ, Liu H, Pellegrini M, Nersissian AM, Gralla EB, Valentine JS, Eisenberg D
Protein Sci 7 545-55 (1998)
PMID: 9541385

Function and Biology Details

Reaction catalysed: 
2 superoxide + 2 H(+) = O(2) + H(2)O(2)
Biochemical function:
Biological process:
Cellular component:
Sequence domains:
Structure domain:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-132494 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Superoxide dismutase [Cu-Zn] Chains: A, B
Molecule details ›
Chains: A, B
Length: 153 amino acids
Theoretical weight: 15.93 KDa
Source organism: Homo sapiens
Expression system: Saccharomyces cerevisiae
UniProt:
  • Canonical: P00441 (Residues: 2-154; Coverage: 99%)
Gene name: SOD1
Sequence domains: Copper/zinc superoxide dismutase (SODC)
Structure domains: Superoxide dismutase, copper/zinc binding domain

Ligands and Environments

2 bound ligands:
Ligand CU 2 x CU
Ligand ZN 2 x ZN
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: RIGAKU RUH2R
Spacegroup: P41
Unit cell:
a: 67.2Å b: 67.2Å c: 83.8Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.202 0.202 0.244
Expression system: Saccharomyces cerevisiae

Quick links

Citations

13 review citations

Copper-zinc superoxide dismutase and amyotrophic lateral sclerosis.
Valentine et al. (2005)
12 more

20 mentions without citation

Misfolded CuZnSOD and amyotrophic lateral sclerosis.
Valentine et al. (2003)
19 more