Recombinant FRDA protein (His tag)

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Recombinant Human Frataxin (FXN) Fragment (aa 89-210) is a high-purity, stable protein product designed for advanced research on mitochondrial iron-sulfur cluster assembly and iron metabolism. This 122-amino acid fragment, derived from the mature form of frataxin, is optimized for solubility and structural integrity, making it an ideal tool for biochemical assays, structural studies, and investigations into Friedreich ataxia mechanisms.

Frataxin is a mitochondrial protein essential for iron-sulfur cluster (ISC) biogenesis, functioning as an activator of persulfide transfer within the core ISC assembly complex. It accelerates sulfur transfer from NFS1 to the scaffold protein ISCU, facilitating de novo synthesis of [2Fe-2S] clusters—a critical step in mitochondrial iron-sulfur protein maturation. Frataxin also binds ferrous ions and may protect against iron-catalyzed oxidative stress, with roles in aconitase reactivation and heme biosynthesis via interaction with ferrochelatase. Mutations in the FXN gene cause Friedreich ataxia, underscoring its importance in neuronal health. The mature form localizes to the mitochondrion, while an extramitochondrial variant modulates cytoplasmic functions, including RNA-binding activity and oxidative stress resistance. This recombinant fragment corresponds to a stable, soluble region of the mature form, enabling focused studies on its functional domains.