QuicKey Pro Human FXN (Frataxin) ELISA Kit (E-OSEL-H0081)

Frataxin protein (FXN) is a highly conserved mitochondrial protein. The mature form of Frataxin acts as a persulfide transferred to the scaffold protein ISCU as an activator, as a component of the core iron-sulfur cluster (ISC) assembly complex, and participates in [2Fe-2S] cluster assembly. It accelerates the transfer of sulfur from NFS1 persulfide intermediate to ISCU and small thiols (such as L-cysteine and glutathione), leading to persulfidation of these thiols and ultimately release of sulfide. It binds ferrous ions and is released from FXN after addition of L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly. The core iron-sulfur cluster (ISC) assembly complex is involved in de novo synthesis of [2Fe-2S] clusters, which is the first step in mitochondrial iron-sulfur protein biogenesis. It may play a role in combating iron-catalyzed oxidative stress through its ability to catalyze oxidation of Fe2+ to Fe3+. It can store large amounts of iron in the form of hydrotalcite mineral through oligomerization. It may function as an iron chaperone, protecting the [4Fe-4S]2+ cluster of apo-enzyme from degradation and promoting enzyme reactivation. It may act as a high-affinity iron-binding partner for FECH, which can both deliver iron to the iron chelatase and mediate the terminal step of mitochondrial heme biosynthesis. Extramitochondrial frataxin regulates the RNA-binding activity of ACO1. It may be involved in cytoplasmic iron-sulfur protein biogenesis. It may contribute to oxidative stress resistance and overall cell survival.