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1.57 A structure of Friedreich's ataxia frataxin variant R165C

Source organism: Homo sapiens
Biochemical function: ferric iron binding
Biological process: iron incorporation into metallo-sulfur cluster
Cellular component: mitochondrion

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EC 1.16.3.1: Ferroxidase

Reaction catalysed:
4 Fe(2+) + 4 H(+) + O(2) = 4 Fe(3+) + 2 H(2)O
Systematic name:
Fe(II):oxygen oxidoreductase
Alternative Name(s):
  • Caeruloplasmin
  • Ceruloplasmin
  • Ferro:O(2) oxidoreductase
  • Ferroxidase I
  • HEPH
  • Hephaestin
  • Iron II:oxygen oxidoreductase
  • Iron oxidase
  • Iron(II):oxygen oxidoreductase

GO terms

Sequence family

Pfam Protein family (Pfam)
PF01491
Domain description: Frataxin-like domain
Occurring in:
  1. Frataxin mature form
The deposited structure of PDB entry 3t3x contains 2 copies of Pfam domain PF01491 (Frataxin-like domain) in Frataxin mature form. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR002908
Domain description: Frataxin/CyaY
Occurring in:
  1. Frataxin mature form
IPR036524
Domain description: Frataxin/CyaY superfamily
Occurring in:
  1. Frataxin mature form
IPR020895
Domain description: Frataxin conserved site
Occurring in:
  1. Frataxin mature form
IPR017789
Domain description: Frataxin
Occurring in:
  1. Frataxin mature form

Structure domain

CATH CATH domain
3.30.920.10
Class: Alpha Beta
Architecture: 2-Layer Sandwich
Topology: Metal Transport, Frataxin; Chain A
Homology: Frataxin/CyaY
Occurring in:
  1. Frataxin mature form
The deposited structure of PDB entry 3t3x contains 2 copies of CATH domain 3.30.920.10 (Metal Transport, Frataxin; Chain A) in Frataxin mature form. Showing 1 copy in chain A.

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