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
- FXN:ISD11:NFS1:ISCUR-HSA-1362404, mitochondrial matrix
- 4 Fe2+CHEBI:29033, Fe, mitochondrial matrix
Outputs
- 2 Iron:FXN:NFS1:ISD11:ISCUR-HSA-1362401, mitochondrial matrix
Catalysed by
- ferrous iron binding activity of FXN:ISD11:NFS1:ISCU [mitochondrial matrix]
Literature
- Human frataxin is an allosteric switch that activates the Fe-S cluster biosynthetic complex Biochemistry, 2010
- Mammalian frataxin: an essential function for cellular viability through an interaction with a preformed ISCU/NFS1/ISD11 iron-sulfur assembly complex PLoS One, 2011
- 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
- Mapping iron binding sites on human frataxin: implications for cluster assembly on the ISU Fe-S cluster scaffold protein J Biol Inorg Chem, 2008
- Assembly and iron-binding properties of human frataxin, the protein deficient in Friedreich ataxia Hum Mol Genet, 2002
- Structure-function analysis of Friedreich's ataxia mutants reveals determinants of frataxin binding and activation of the Fe-S assembly complex Biochemistry, 2011