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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Frataxin binds iron

FXN:ISD11:NFS1:ISCU + 4 Fe2+ → 2 Iron:FXN:NFS1:ISD11:ISCU

Reactome
R-HSA-1362416
Compartment
mitochondrial matrix
Rhea
None recorded

Inputs

Outputs

Catalysed by

  • ferrous iron binding activity of FXN:ISD11:NFS1:ISCU [mitochondrial matrix]

Literature

  1. Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex Biochemistry, 2010
  2. Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex PLoS One, 2011
  3. Iron-sulfur cluster biosynthesis. Characterization of frataxin as an iron donor for assembly of [2Fe-2S] clusters in ISU-type proteins J Am Chem Soc, 2003
  4. Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe-S cluster scaffold protein J Biol Inorg Chem, 2008
  5. Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia Hum Mol Genet, 2002
  6. Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex Biochemistry, 2011