Anti-FXN / Frataxin Rabbit Polyclonal Antibody for WB, IHC-P, IF/ICC, ELISA - Q16595

Core Product Specifications and Parameters

Biological Background: Frataxin (FXN) Function and Localization

• Frataxin is an essential component of the mitochondrial iron-sulfur cluster (ISC) assembly machinery, where it functions as an activator of persulfide transfer from the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) to the scaffold protein ISCU, facilitating [2Fe-2S] cluster synthesis (PubMed:12785837, PubMed:24971490).
  Related references: PMID:12785837, PMID:24971490
• It binds ferrous iron and is released upon addition of L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly (PubMed:29576242).
  Related references: PMID:29576242
• The oligomeric form of frataxin exhibits in vitro ferroxidase activity, catalyzing Fe²⁺ oxidation to Fe³⁺, potentially protecting against iron-catalyzed oxidative stress (PubMed:15641778).
  Related references: PMID:15641778
• Frataxin may also store iron as a ferrihydrite mineral via oligomerization, though the physiological relevance remains debated (PubMed:11823441, PubMed:12755598).
  Related references: PMID:11823441, PMID:12755598
• It functions as an iron chaperone, protecting the [4Fe-4S]²⁺ cluster of aconitase from disassembly and promoting enzyme reactivation (PubMed:15247478).
  Related references: PMID:15247478
• Frataxin may serve as a high-affinity iron donor for ferrochelatase (FECH), mediating the terminal step in mitochondrial heme biosynthesis (PubMed:15123683, PubMed:16239244).
  Related references: PMID:15123683, PMID:16239244
• Beyond mitochondria, frataxin is found in the cytosol where it modulates the RNA-binding activity of ACO1 (iron regulatory protein 1) and may participate in cytoplasmic iron-sulfur protein biogenesis (PubMed:20053667, PubMed:16091420). It contributes to oxidative stress resistance and cell survival (PubMed:16608849).
  Related references: PMID:20053667, PMID:16091420, PMID:16608849
• Frataxin is predominantly localized in mitochondria, with a minor cytosolic pool, and is expressed highly in heart, peripheral blood lymphocytes, and dermal fibroblasts. The gene is subject to alternative splicing and is associated with Friedreich's ataxia, characterized by GAA triplet repeat expansion.

Experimental Guidance and Technical Tips

• The immunogen corresponds to amino acids 42–210 of human frataxin (mature protein), a region conserved across human, mouse, and rat, consistent with the reported cross-reactivity.
• For Western blotting, the predicted molecular weight of the mature frataxin is ~23 kDa; the precursor form is ~27 kDa. Optimize blocking (e.g., 5% nonfat milk or BSA) and antibody dilution for your sample type.
• For immunohistochemistry (IHC) and immunofluorescence (IF), antigen retrieval conditions (citrate buffer pH 6.0 or EDTA pH 9.0) may require optimization; include appropriate negative controls.
• In ELISA, consider using recombinant frataxin or cell lysates as coating antigens and validate linearity and specificity.
• As a polyclonal antibody, it recognizes multiple epitopes, which can enhance detection sensitivity but may also increase background; titrate the antibody carefully in each application.
• Always include appropriate positive and negative controls, and validate the antibody in your specific experimental system.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio is your one-stop sourcing partner for IVD raw materials, offering validated antibody pairs, recombinant proteins, and comprehensive technical support from concept to clinic. This anti-Frataxin polyclonal antibody exemplifies our commitment to providing reliable tools for mitochondrial research, iron metabolism, and Friedreich’s ataxia. Our platform ensures stable supply and expert consultation to accelerate your diagnostic development.