Anti-Frataxin Recombinant Antibody (N191/6) (HS777013)

Anti-Frataxin Antibody (N191/6) (abinScience Cat# HS777013, RRID:AB_3728444)
_x005F_x000D_ Copy Citation

Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA.

Frataxin, mitochondrial (FXN) is a ~23 kDa protein. Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly. Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release. Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly. The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner.