Anti-Frataxin (FXN) Polyclonal Antibody for WB - Q16595

Core Product Specifications and Parameters

Biological Background: Frataxin Function and Localization

• Encoded by the FXN gene (also known as FRDA or X25), frataxin is a 23-kDa mitochondrial protein that is highly conserved. Expansions of an intronic GAA triplet repeat cause Friedreich ataxia, a neurodegenerative disease.
• Frataxin is a key component of the core iron-sulfur cluster (ISC) assembly complex in mitochondria, where it activates persulfide transfer from the cysteine desulfurase complex NFS1:LYRM4:NDUFAB1 to the scaffold protein ISCU, enabling de novo [2Fe-2S] cluster synthesis. Related references: PMID:12785837 PMID:24971490
• It binds ferrous iron and is released upon addition of L-cysteine and reduced FDX2, facilitating [2Fe-2S] cluster assembly. The mature cluster is then transferred to chaperones like HSCB, HSPA9, and GLRX5. Related references: PMID:29576242
• Beyond mitochondrial ISC biogenesis, frataxin may protect against oxidative stress by catalyzing Fe²⁺ to Fe³⁺ oxidation (ferroxidase activity) in its oligomeric form, and it can store iron as a ferrihydrite mineral. Related references: PMID:15641778 PMID:11823441
• Frataxin may act as an iron chaperone, delivering iron to ferrochelatase (FECH) for heme biosynthesis and protecting aconitase’s [4Fe-4S] cluster from disassembly. Related references: PMID:15123683 PMID:15247478
• The protein is predominantly located in the mitochondrial matrix but is also found in the cytoplasm, where it may participate in cytosolic iron-sulfur protein biogenesis and modulate ACO1 RNA-binding activity. Related references: PMID:20053667
• Alternative splicing generates multiple isoforms, and the protein undergoes proteolytic processing to remove the mitochondrial targeting sequence.
• Frataxin is expressed in metabolically active tissues such as heart, peripheral blood lymphocytes, and dermal fibroblasts.

Experimental Guidance and Technical Tips

• The immunogen spans residues 42–210, covering the mature processed form of frataxin, which is expected to detect endogenous protein in mitochondrial and cytosolic fractions.
• For Western blot (WB), a band near 23 kDa is anticipated. Given the polyclonal nature of the antibody, multiple bands may appear from isoforms or processing intermediates; validate under reducing conditions.
• In immunohistochemistry (IHC-P) and immunofluorescence (IF/ICC), consider antigen retrieval and mitochondrial co-staining to confirm localization.
• The antibody has predicted cross-reactivity with mouse and rat frataxin based on high sequence homology, but performance should be verified in your model system.
• For ELISA, use the antibody as a capture or detection reagent; pairing with a matched monoclonal may improve assay specificity.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio offers validated antibody pairs, optimized polyclonal antibodies like this anti-Frataxin pAb, and bulk ancillary reagents to support diagnostic assay development and mitochondrial research. From single-ingredient sourcing to comprehensive raw-material packages, we bridge the gap between concept and clinic. Contact us to discuss your requirements for iron-sulfur cluster targets or rare disease markers — we deliver reliable, scalable IVD solutions.