› 3s5d

X-ray diffraction
1.5Å resolution

Crystal structure of human frataxin variant W155A

Released:
DOI: 10.2210/pdb3s5d/pdb
PDB entry 3s5d coloured by chain, front view.
PDB entry 3s5d coloured by chain, side view.
PDB entry 3s5d coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Friedreich's ataxia variants I154F and W155R diminish frataxin-based activation of the iron-sulfur cluster assembly complex.
Tsai CL, Bridwell-Rabb J, Barondeau DP
Biochemistry 50 6478-87 (2011)
PMID: 21671584

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:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-172519 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Extramitochondrial frataxin Chain: A
Molecule details ›
Chain: A
Length: 129 amino acids
Theoretical weight: 14.08 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli BL21(DE3)
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 1 x SO4
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-D
Spacegroup: C2
Unit cell:
a: 87.49Å b: 32.26Å c: 44.81Å
α: 90° β: 91.36° γ: 90°
R-values:
R R work R free
0.17 0.169 0.192
Expression system: Escherichia coli BL21(DE3)

Quick links

Citations

5 review citations

Emerging critical roles of Fe-S clusters in DNA replication and repair.
Fuss et al. (2015)
4 more

2 mentions without citation

Trinucleotide repeats: a structural perspective.
Almeida et al. (2013)
1 more