Anti-Frataxin/FXN Rabbit Monoclonal Antibody for WB, IF/ICC, ELISA - Q16595

Core Product Specifications and Parameters

Biological Background: Frataxin/FXN Function and Localization

• Frataxin (FXN), also known as Friedreich ataxia protein, is a mitochondrial protein encoded by the FXN (FRDA, X25) gene. Mutations leading to GAA triplet repeat expansion cause Friedreich ataxia, a neurodegenerative disorder.
• Frataxin is a component of the core iron-sulfur cluster (ISC) assembly complex, where it functions as an activator of persulfide transfer from the cysteine desulfurase complex NFS1:LYRM4:NDUFAB1 to the scaffold protein ISCU, accelerating sulfur transfer and facilitating [2Fe-2S] cluster assembly (PubMed:12785837, PubMed:24971490). It binds ferrous ion and is released upon the addition of L-cysteine and reduced FDX2 during cluster assembly (PubMed:29576242). Related references: PMID:12785837 PMID:24971490 PMID:29576242
• The oligomeric form of frataxin exhibits ferroxidase activity, catalyzing the oxidation of Fe²⁺ to Fe³⁺, which may protect against iron-catalyzed oxidative stress (PubMed:15641778). Related references: PMID:15641778
• Frataxin can store iron as a ferrihydrite mineral via oligomerization (PubMed:11823441, PubMed:12755598) and may serve as an iron chaperone, protecting the aconitase [4Fe-4S] cluster from disassembly (PubMed:15247478) and delivering iron to ferrochelatase for heme biosynthesis (PubMed:15123683, PubMed:16239244). Related references: PMID:11823441 PMID:12755598 PMID:15247478 PMID:15123683 PMID:16239244
• In the cytoplasm, frataxin modulates the RNA-binding activity of ACO1 (PubMed:20053667), and may contribute to cytoplasmic iron-sulfur protein biogenesis (PubMed:16091420) and cellular resistance to oxidative stress (PubMed:16608849). Related references: PMID:20053667 PMID:16091420 PMID:16608849
• Predominantly localized to the mitochondrion, frataxin is also detected in the cytoplasm and cytosol.
• Expressed in the heart, peripheral blood lymphocytes, and dermal fibroblasts.

Experimental Guidance and Technical Tips

• The immunogen spans amino acids 42–210, covering the mature frataxin region after mitochondrial transit peptide cleavage. Consider validating detection of both precursor and mature forms in mitochondrial vs. cytosolic fractions.
• For Western blotting, a band near 23 kDa corresponds to mature frataxin; precursor forms may appear slightly higher. Use reducing conditions and appropriate molecular weight markers.
• In immunofluorescence (IF/ICC), expect a mitochondrial staining pattern. Co‑staining with a mitochondrial marker is advisable to confirm localization.
• ELISA applications may benefit from epitope mapping to determine compatibility with capture/detection pairs in sandwich assay development.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio stands as a premier one-stop sourcing partner for IVD raw materials, providing diagnostic manufacturers and laboratories with comprehensive access to validated antibody pairs, optimized monoclonal antibodies like this anti-Frataxin/FXN clone, and bulk ancillary reagents. Whether you are developing assays for mitochondrial research or Friedreich ataxia diagnostics, CamelBio facilitates seamless sourcing from concept to clinic, even for rare disease targets.