3.0
Summary
| Name |  FXNView Modifications |
| Full Name | Frataxin, mitochondrial |
| Synonyms | Friedreich ataxia protein, Fxn | FRDA, X25 |
| Primary ID | Q16595 |
| Links |  -  -  |
| Type | protein |
| Relations | 2 |
| Function | Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) 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 Fe(2+) to Fe(3+); 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. |
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