Frataxin transfers Fe2+ to ACO2
ACO2:3Fe-4S + 2 Iron:FXN → ACO2:4Fe-4S + FXN(81-210) + Fe2+
- Reactome
- R-HSA-9854405
- Compartment
- mitochondrial matrix
- Rhea
- None recorded
Inputs
- ACO2:3Fe-4SR-HSA-9854373, mitochondrial matrix
- 2 Iron:FXNR-HSA-1362402, mitochondrial matrix
Outputs
- ACO2:4Fe-4SR-HSA-9854344, mitochondrial matrix
- FXN(81-210)R-HSA-1362390, mitochondrial matrix
- Fe2+CHEBI:29033, Fe, mitochondrial matrix
Catalysed by
- iron chaperone activity of 2 Iron:FXN [mitochondrial matrix]
Literature
- Redox sensitive human mitochondrial aconitase and its interaction with frataxin: In vitro and in silico studies confirm that it takes two to tango Free Radic Biol Med, 2023
- Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity Science, 2004
- Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes Ann Neurol, 1999