Anti-Frataxin/FXN Rabbit Monoclonal Antibody [KD Validated] for WB, IF/ICC, IHC-P, ELISA - Q16595

Core Product Specifications and Parameters

Biological Background: Frataxin Function and Localization

• Frataxin (FXN) is a mitochondrial protein that functions as a critical activator of persulfide transfer to the scaffold protein ISCU within the core iron-sulfur cluster (ISC) assembly complex, essential for de novo [2Fe-2S] cluster biogenesis.
  Related references: PMID:12785837, PMID:24971490

• The core ISC complex, consisting of NFS1:LYRM4:NDUFAB1 and ISCU, produces persulfide that is delivered to ISCU in an FXN-dependent manner; the complex is then stabilized by FDX2, enabling [2Fe-2S] cluster assembly and transfer to chaperones (HSCB, HSPA9, GLRX5) (By similarity).

• FXN binds ferrous ion (Fe²⁺) and is released upon addition of L-cysteine and reduced FDX2 during cluster assembly; it accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and small thiols like L-cysteine and glutathione, promoting persulfuration and sulfide release.
  Related references: PMID:24971490, PMID:29576242

• The oligomeric form of FXN exhibits in vitro ferroxidase activity, catalyzing the oxidation of Fe²⁺ to Fe³⁺, which may protect against iron-catalyzed oxidative stress.
  Related references: PMID:15641778

• FXN may function as an iron storage protein, forming ferrihydrite mineral via oligomerization, and as an iron chaperone that protects aconitase’s [4Fe-4S]²⁺ cluster from disassembly, promoting enzyme reactivation.
  Related references: PMID:11823441, PMID:12755598, PMID:15247478

• It acts as a high-affinity iron binding partner for ferrochelatase (FECH), delivering iron and mediating the terminal step of mitochondrial heme biosynthesis.
  Related references: PMID:15123683, PMID:16239244

• FXN modulates the RNA-binding activity of ACO1 and contributes to cytoplasmic iron-sulfur protein biogenesis, oxidative stress resistance, and overall cell survival.
  Related references: PMID:20053667, PMID:16091420, PMID:16608849

• Subcellular localization includes mitochondria and the cytosol; tissue expression is observed in heart, peripheral blood lymphocytes, and dermal fibroblasts. Mutations in FXN are linked to Friedreich ataxia, a triplet repeat expansion disorder.

Experimental Guidance and Technical Tips

• The immunogen (amino acids 42–210 of human FXN) covers the mature protein portion, expected to detect both full-length and processed mitochondrial forms; in WB, consider the predicted precursor band at ~23 kDa and potential smaller cleaved products.
• KD (knock-down) validation of this antibody is indicated, suggesting target specificity; however, researchers should validate expression in their cell system of interest (e.g., HeLa, HepG2, or fibroblast lines).
• For IF/ICC and IHC-P, it may be necessary to optimize fixation/permeabilization protocols to access mitochondrial and cytosolic antigens; consider validating with known positive control tissues (e.g., heart, skin).
• As a rabbit monoclonal antibody, it offers consistent batch performance and is suitable for ELISA-based capture or detection workflows; pair with appropriate detection reagents and validate in the relevant sample matrix.

CamelBio: Your One-Stop Sourcing Bridge

CamelBio supports diagnostic manufacturers and research labs with a comprehensive portfolio of IVD raw materials, including validated antibody pairs, recombinant proteins, and ancillary reagents. For mitochondrial iron-sulfur cluster biogenesis and Friedreich ataxia research, we offer optimized antibodies, bulk ordering options, and rare-target sourcing to accelerate your assay development. Connect with our technical team to explore custom sourcing solutions that bridge discovery and clinical application.