› 3t3j

X-ray diffraction
1.7Å resolution

1.70 A structure of Friedreich's ataxia frataxin variant N146K

Released:
DOI: 10.2210/pdb3t3j/pdb
PDB entry 3t3j coloured by chain, front view.
PDB entry 3t3j coloured by chain, side view.
PDB entry 3t3j 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 Chain: A
Molecule details ›
Chain: A
Length: 129 amino acids
Theoretical weight: 14.21 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

No bound ligands
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-D
Spacegroup: P21
Unit cell:
a: 30.47Å b: 45.82Å c: 38.9Å
α: 90° β: 97.36° γ: 90°
R-values:
R R work R free
0.214 0.214 0.245
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

2 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)
1 more