Frataxin transfers Fe2+ to ACO2

Stable Identifier
R-HSA-9854405
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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Frataxin (FXN) is an iron chaperone that acts as a protein partner of ACO2 not only during Fe–S maturation but also during the repairing of inactivated [3Fe–4S]+-ACO2. It donates one Fe atom to reactivate ACO2 with a full [4Fe-4S](2+) cluster (Bulteau et al., 2004; Mansilla et al., 2023). Mutations in FXN can lead to Friedreich ataxia (FXAD, MIM:229300; see e.g. Cossee et al., 1999).
Literature References
PubMed ID Title Journal Year
15247478 Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

Isaya, G, Ikeda-Saito, M, O'Neill, HA, Szweda, LI, Kennedy, MC, Bulteau, AL

Science 2004
36738801 Redox sensitive human mitochondrial aconitase and its interaction with frataxin: In vitro and in silico studies confirm that it takes two to tango

Tórtora, V, Santos, J, Chiribao, ML, Mansilla, S, Castro, I, Pignataro, F, Robello, C, Sastre, S, Castro, L, Zeida, A

Free Radic Biol Med 2023
9989622 Friedreich's ataxia: point mutations and clinical presentation of compound heterozygotes

Dahl, N, De Michele, G, Dürr, A, Cocozza, S, Kohlschutter, A, Labuda, M, Müller, U, Poirier, J, Mandel, JL, Koenig, M, Trouillas, P, Allinson, P, Tammaro, A, Schmitt, M, Cossée, M, Cavalcanti, F, Kostrzewa, M, Pandolfo, M, Filla, A, Brice, A, Gustavson, KH, Montermini, L, Nivelon-Chevallier, A

Ann Neurol 1999
Participants
Participates
Catalyst Activity

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

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