› 3gqf

X-ray diffraction
2.2Å resolution

Structural and Biophysical Properties of the Pathogenic SOD1 Variant H46R/H48Q

Released:
DOI: 10.2210/pdb3gqf/pdb
PDB entry 3gqf coloured by chain, front view.
PDB entry 3gqf coloured by chain, side view.
PDB entry 3gqf coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structural and biophysical properties of the pathogenic SOD1 variant H46R/H48Q.
Winkler DD, Schuermann JP, Cao X, Holloway SP, Borchelt DR, Carroll MC, Proescher JB, Culotta VC, Hart PJ
Biochemistry 48 3436-47 (2009)
PMID: 19227972

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 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, C, D, E, F
Molecule details ›
Chains: A, B, C, D, E, F
Length: 153 amino acids
Theoretical weight: 15.84 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 ZN 6 x ZN
Ligand CA 5 x CA
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: RIGAKU MICROMAX-007 HF
Spacegroup: C2221
Unit cell:
a: 112.576Å b: 194.331Å c: 143.179Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.207 0.205 0.242
Expression system: Saccharomyces cerevisiae

Quick links

Citations

4 review citations

Copper metallochaperones.
Robinson et al. (2010)
3 more

2 mentions without citation

Structural Properties and Interaction Partners of Familial ALS-Associated SOD1 Mutants.
Huai et al. (2019)
1 more