Frataxin, Mitochondrial

E5308Hu

Sandwich

48T,96T

7.81ng/L

15-3000ng/L

Serum, plasma, cell culture supernates

Human

2-8ºC

1h 30m

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.

23,135

2395

d-FXN;Frataxin intermediate form;Frataxin mature form;Frataxin(56-210;Frataxin(78-210;Frataxin(81-210;Frataxin, mitochondrial;Friedreich ataxia protein;FXN;i-FXN;m56-FXN;m78-FXN;m81-FXN;

FXN

Tags & Cell Markers

FXN