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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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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

Outputs

Catalysed by

  • iron chaperone activity of 2 Iron:FXN [mitochondrial matrix]

Literature

  1. 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
  2. Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity Science, 2004
  3. Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes Ann Neurol, 1999