| Field | Specification |
|---|---|
| Mfr No | |
| Clonality | |
| Host | |
| Immunogen | E.coli-derived human FXN recombinant protein (Position: Q20-A210) was used as the immunogen for the FXN antibody. |
| Isotype | |
| Product Type | |
| Purity | |
| Reactivity | |
| Storage | |
| Target | |
| UniProt # |
Overview
FXN Antibody / Frataxin is a anti-FXN Rabbit antibody Polyclonal (rabbit origin) supplied in Lyophilized format. Recommended for workflows such as Western blot (WB), Immunohistochemistry (IHC), Flow cytometry (FACS), ELISA with listed reactivity in Human. Reported localization: Cytoplasm, Mitochondria.
Key elements and design rationale
- Target: FXN
- Antibody details: Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG
- Format: Lyophilized
- Applications (as listed): WB, IHC, FACS, ELISA
Biological background
FXN acts as an iron chaperone that delivers ferrous iron (Fe2+) to scaffold proteins such as ISCU for the assembly of Fe-S clusters. These clusters serve as cofactors for numerous mitochondrial enzymes, including aconitase and complex I-III components of the respiratory chain. By regulating mitochondrial iron utilization, FXN prevents toxic iron accumulation and the generation of reactive oxygen species (ROS). Co-localization studies demonstrate FXN interaction with ISCU and NFS1 within mitochondrial nucleoids, coordinating Fe-S cluster synthesis and incorporation into target proteins.
Structurally, Frataxin is a small alpha-beta fold protein with a conserved acidic ridge that binds iron ions. It belongs to the mitochondrial Fe-S cluster assembly machinery family. FXN also associates with chaperones and proteases such as HSP60 and Lon protease, maintaining protein stability and turnover in the mitochondrial matrix. It undergoes processing from a cytosolic precursor into a mature mitochondrial form via the mitochondrial targeting sequence at its N-terminus.
Functionally, FXN is crucial for cellular respiration, antioxidant defense, and metabolic balance. It supports the activity of Fe-S-dependent enzymes in the electron transport chain and modulates mitochondrial redox status. In neurons, FXN ensures energy supply and protects against oxidative stress, while in cardiac tissue, it maintains contractile efficiency by preserving mitochondrial integrity. Developmentally, FXN expression peaks during embryogenesis and early postnatal stages, coinciding with rapid mitochondrial biogenesis in growing tissues.
Loss-of-function mutations in FXN cause Friedreich's ataxia, an autosomal recessive neurodegenerative disease characterized by impaired motor coordination, cardiomyopathy, and diabetes. The deficiency leads to mitochondrial iron overload, defective Fe-S cluster assembly, and increased oxidative stress. Pathway involvement includes Fe-S cluster biosynthesis, oxidative phosphorylation, and cellular stress response. In cancer research, FXN downregulation is associated with altered metabolic reprogramming and mitochondrial dysfunction.
Immunohistochemical staining using FXN antibody shows mitochondrial localization in neurons, cardiac myocytes, and hepatocytes. The FXN antibody from
Research relevance and current trends
- Connecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).
- Considering isoforms and post-translational regulation when interpreting protein-level changes.
- Comparing results across species and model systems with matched controls.
Common research applications
- Western blotting: compare relative abundance and activation-state changes across conditions.
- Immunohistochemistry: map target signal in tissue context and compare regions/phenotypes.
- Flow cytometry: quantify target-positive populations and signal shifts at single-cell resolution.
- ELISA: support antibody-based quantification in assay formats where applicable.
Interpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.
Notes for experimental interpretation
- Signal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.
- Species differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.
Antibody notes: Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.
Customization & Add-ons: Can’t find the antibody you need—or require a custom format for your assay? We can help you source the best match or support custom antibody solutions for diverse research needs, including species and isotype selection, conjugations and labeling (e.g., HRP/AP, biotin, fluorophores), purification grade options (Protein A/G, affinity purified), formulation preferences (buffer selection, carrier-free, glycerol-free), custom concentrations and aliquoting, low-endotoxin options for cell-based work, and application-focused QC/validation support (project dependent). Click Talk to a Scientist to submit a request, email us at support@biohippo.com, or explore our Research Services for additional support—our team will follow up with feasibility details and next steps.
