Frataxin and mitochondrial FeS cluster biogenesis

Timothy L. Stemmler, Emmanuel Lesuisse, Debkumar Pain, Andrew Dancis

Research output: Contribution to journalShort surveypeer-review

122 Scopus citations


Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell. They are synthesized by a multistep and multisubunit mitochondrial machinery that includes the scaffold protein Isu for assembling a protein-bound FeS cluster intermediate. Frataxin interacts with Isu, iron, and the cysteine desulfurase Nfs1, which supplies sulfide, thus placing it at the center of mitochondrial FeS cluster biosynthesis.

Original languageEnglish (US)
Pages (from-to)26737-26743
Number of pages7
JournalJournal of Biological Chemistry
Issue number35
StatePublished - Aug 27 2010

All Science Journal Classification (ASJC) codes

  • Biochemistry
  • Molecular Biology
  • Cell Biology


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