› 6nzu

Electron Microscopy
3.2Å resolution

Structure of the human frataxin-bound iron-sulfur cluster assembly complex

Released:
DOI: 10.2210/pdb6nzu/pdb
PDB entry 6nzu coloured by chain, front view.
PDB entry 6nzu coloured by chain, side view.
PDB entry 6nzu coloured by chain, top view.
Source organisms:
Primary publication:
Structure of the human frataxin-bound iron-sulfur cluster assembly complex provides insight into its activation mechanism.
Fox NG, Yu X, Feng X, Bailey HJ, Martelli A, Nabhan JF, Strain-Damerell C, Bulawa C, Yue WW, Han S
Nat Commun 10 2210 (2019)
PMID: 31101807
Related structures: EMD-0560

Function and Biology Details

Reactions catalysed: 
(1a) L-cysteine + [enzyme]-cysteine = L-alanine + [enzyme]-S-sulfanylcysteine
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:
hetero decamer (preferred)
PDBe Complex ID:
PDB-CPX-167173 (preferred)
Entry contents:
5 distinct polypeptide molecules
Macromolecules (5 distinct):
Cysteine desulfurase Chains: A, E
Molecule details ›
Chains: A, E
Length: 403 amino acids
Theoretical weight: 44.85 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'
UniProt:
  • Canonical: Q9Y697 (Residues: 55-457; Coverage: 88%)
Gene names: HUSSY-08, NFS1, NIFS
Sequence domains: Aminotransferase class-V
LYR motif-containing protein 4 Chains: B, F
Molecule details ›
Chains: B, F
Length: 92 amino acids
Theoretical weight: 10.85 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'
UniProt:
  • Canonical: Q9HD34 (Residues: 1-91; Coverage: 100%)
Gene names: C6orf149, CGI-203, ISD11, LYRM4
Sequence domains: Complex 1 protein (LYR family)
Acyl carrier protein Chains: C, G
Molecule details ›
Chains: C, G
Length: 74 amino acids
Theoretical weight: 8.25 KDa
Source organism: Escherichia coli
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'
Iron-sulfur cluster assembly enzyme ISCU Chains: D, H
Molecule details ›
Chains: D, H
Length: 124 amino acids
Theoretical weight: 13.41 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'
UniProt:
  • Canonical: Q9H1K1 (Residues: 34-157; Coverage: 74%)
Gene names: ISCU, NIFUN
Sequence domains: NifU-like N terminal domain
Structure domains: Sufe protein. Chain: A
Frataxin mature form Chains: I, J
Molecule details ›
Chains: I, J
Length: 132 amino acids
Theoretical weight: 14.5 KDa
Source organism: Homo sapiens
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'
UniProt:
  • Canonical: Q16595 (Residues: 81-210; Coverage: 62%)
Gene names: FRDA, FXN, X25
Sequence domains: Frataxin-like domain
Structure domains: Frataxin/CyaY

Ligands and Environments


Cofactor: Ligand PLP 2 x PLP
2 bound ligands:
Ligand 8Q1 2 x 8Q1
Ligand ZN 2 x ZN
No modified residues

Experiments and Validation Details

Entry percentile scores
Resolution: 3.2Å
Relevant EMDB volumes: EMD-0560
Expression system: Escherichia coli 'BL21-Gold(DE3)pLysS AG'

Quick links

Citations

16 review citations

Thioredoxin and Glutaredoxin Systems as Potential Targets for the Development of New Treatments in Friedreich's Ataxia.
Seco-Cervera et al. (2020)
15 more

11 mentions without citation

Outlining the Complex Pathway of Mammalian Fe-S Cluster Biogenesis.
Maio et al. (2020)
10 more