Iron-sulphur cluster biogenesis and mitochondrial iron homeostasis

Nat Rev Mol Cell Biol. 2005 Apr;6(4):345-51. doi: 10.1038/nrm1620.


Iron-sulphur clusters are important cofactors for proteins that are involved in many cellular processes, including electron transport, enzymatic catalysis and regulation. The enzymes that catalyse the formation of iron-sulphur clusters are widely conserved from bacteria to humans. Recent studies in model systems and humans reveal that iron-sulphur proteins have important roles in mitochondrial iron homeostasis and in the pathogenesis of the human disease Friedreich ataxia.

Publication types

  • Review

MeSH terms

  • Anemia, Sideroblastic
  • Animals
  • Evolution, Molecular
  • Friedreich Ataxia / pathology
  • Friedreich Ataxia / therapy
  • Homeostasis
  • Humans
  • Iron / metabolism*
  • Iron Overload / metabolism
  • Iron-Sulfur Proteins / genetics
  • Iron-Sulfur Proteins / metabolism*
  • Mice
  • Mitochondria / metabolism*
  • Protein Structure, Tertiary
  • Yeasts / metabolism


  • Iron-Sulfur Proteins
  • Iron