The role of mitochondria in cytosolic-nuclear iron–sulfur protein biogenesis and in cellular iron regulation

Curr Opin Microbiol. 2014 Dec;22:111-9. doi: 10.1016/j.mib.2014.09.015.


Mitochondria are indispensable in eukaryotes because of their function in the maturation of cytosolic and nuclear iron–sulfur proteins that are essential for DNA synthesis and repair, tRNA modification, and protein translation. The mitochondrial Fe/S cluster assembly machinery not only generates the organelle's iron–sulfur proteins, but also extra-mitochondrial ones. Biogenesis of the latter proteins requires the mitochondrial ABC transporter Atm1 that exports a sulfur-containing compound in a glutathione-dependent fashion. The process is further assisted by the cytosolic iron–sulfur protein assembly machinery. Here, we discuss the knowns and unknowns of the mitochondrial export process that is also crucial for signaling the cellular iron status to the regulatory systems involved in the maintenance of cellular iron homeostasis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • ATP-Binding Cassette Transporters / metabolism
  • Biological Transport
  • Cell Nucleus / metabolism
  • Cytosol / metabolism
  • Glutathione / metabolism
  • Homeostasis
  • Iron / metabolism*
  • Iron-Sulfur Proteins / metabolism*
  • Mitochondria / metabolism*
  • Oxidoreductases / metabolism
  • Protein Binding


  • ATP-Binding Cassette Transporters
  • Iron-Sulfur Proteins
  • Iron
  • Oxidoreductases
  • Glutathione