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Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.
Gene Name: | frataxin |
Synonyms: | FXN, CyaY, Frataxin, mitochondrial, Friedreich ataxia protein, FARR, Friedreich ataxia, FA, FRDA, X25, Frataxin |
Target Sequences: | NM_000144 NP_000135.2 Q16595 |
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