Frataxin, mitochondrial (FXN)

Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by providing Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress by its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity.

May be able to store considerable amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological significance is unsure as reports are conflicting and the purpose has just been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.