› 3t3t

X-ray diffraction
1.38Å resolution

1.38 A structure of human frataxin variant Q148G

Released:
DOI: 10.2210/pdb3t3t/pdb
PDB entry 3t3t coloured by chain, front view.
PDB entry 3t3t coloured by chain, side view.
PDB entry 3t3t coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex.
Bridwell-Rabb J, Winn AM, Barondeau DP
Biochemistry 50 7265-74 (2011)
PMID: 21776984

Function and Biology Details

Reaction catalysed: 
4 Fe(2+) + 4 H(+) + O(2) = 4 Fe(3+) + 2 H(2)O
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-172517 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Frataxin mature form Chains: A, B, C, D
Molecule details ›
Chains: A, B, C, D
Length: 129 amino acids
Theoretical weight: 14.12 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli
UniProt:
  • Canonical: Q16595 (Residues: 82-210; Coverage: 61%)
Gene names: FRDA, FXN, X25
Sequence domains: Frataxin-like domain
Structure domains: Frataxin/CyaY

Ligands and Environments

1 bound ligand:
Ligand SO4 4 x SO4
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: SSRL BEAMLINE BL11-1
Spacegroup: P21
Unit cell:
a: 46.41Å b: 68.56Å c: 89.97Å
α: 90° β: 93.23° γ: 90°
R-values:
R R work R free
0.199 0.198 0.227
Expression system: Escherichia coli

Quick links

Citations

13 review citations

Biogenesis of iron-sulfur clusters in mammalian cells: new insights and relevance to human disease.
Rouault TA. (2012)
12 more

3 mentions without citation

Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex.
Bridwell-Rabb et al. (2011)
2 more