{"product_id":"fxn-antibody-frataxin-bha17136249","title":"FXN Antibody \/ Frataxin","description":"\u003ch2\u003eOverview\u003c\/h2\u003e\u003cp\u003eFXN 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.\u003c\/p\u003e\u003ch2\u003eKey elements and design rationale\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eTarget:\u003c\/strong\u003e FXN\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eAntibody details:\u003c\/strong\u003e Rabbit, Polyclonal (rabbit origin), isotype Rabbit IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFormat:\u003c\/strong\u003e Lyophilized\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eApplications (as listed):\u003c\/strong\u003e WB, IHC, FACS, ELISA\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eBiological background\u003c\/h2\u003e\u003cdiv\u003eFXN antibody detects Frataxin, a mitochondrial matrix protein encoded by the FXN gene on chromosome 9q21.11. Frataxin plays a fundamental role in iron-sulfur (Fe-S) cluster biogenesis, iron homeostasis, and oxidative stress protection. It is ubiquitously expressed but highly abundant in metabolically active tissues such as heart, skeletal muscle, pancreas, and neurons, where mitochondrial energy metabolism is critical. FXN belongs to the frataxin family of mitochondrial proteins and is essential for the proper function of enzymes involved in oxidative phosphorylation and the tricarboxylic acid (TCA) cycle.\u003cbr\u003e\u003cbr\u003eFXN 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.\u003cbr\u003e\u003cbr\u003eStructurally, 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.\u003cbr\u003e\u003cbr\u003eFunctionally, 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.\u003cbr\u003e\u003cbr\u003eLoss-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.\u003cbr\u003e\u003cbr\u003eImmunohistochemical staining using FXN antibody shows mitochondrial localization in neurons, cardiac myocytes, and hepatocytes. The FXN antibody from\u003c\/div\u003e\u003ch2\u003eResearch relevance and current trends\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eConnecting protein-level changes to phenotype using orthogonal readouts (genetic perturbation, transcriptomics, imaging).\u003c\/li\u003e\n\u003cli\u003eConsidering isoforms and post-translational regulation when interpreting protein-level changes.\u003c\/li\u003e\n\u003cli\u003eComparing results across species and model systems with matched controls.\u003c\/li\u003e\n\u003c\/ul\u003e\u003ch2\u003eCommon research applications\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003e\n\u003cstrong\u003eWestern blotting:\u003c\/strong\u003e compare relative abundance and activation-state changes across conditions.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eImmunohistochemistry:\u003c\/strong\u003e map target signal in tissue context and compare regions\/phenotypes.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eFlow cytometry:\u003c\/strong\u003e quantify target-positive populations and signal shifts at single-cell resolution.\u003c\/li\u003e\n\u003cli\u003e\n\u003cstrong\u003eELISA:\u003c\/strong\u003e support antibody-based quantification in assay formats where applicable.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003eInterpret changes in signal alongside appropriate controls and, when relevant, in parallel with total-protein or pathway readouts.\u003c\/p\u003e\u003ch2\u003eNotes for experimental interpretation\u003c\/h2\u003e\u003cul\u003e\n\u003cli\u003eSignal can reflect expression level, isoform composition, and post-translational state; interpret results in the context of your model system and stimuli.\u003c\/li\u003e\n\u003cli\u003eSpecies differences and sample matrices can influence epitope recognition; prioritize matched controls and orthogonal confirmation when feasible.\u003c\/li\u003e\n\u003c\/ul\u003e\u003cp\u003e\u003cstrong\u003eAntibody notes:\u003c\/strong\u003e Polyclonal antibodies recognize multiple epitopes, which can broaden the epitope footprint and may increase sensitivity in some contexts.\u003c\/p\u003e\u003c!-- Sources (internal): - UniProt search — UniProt — https:\/\/www.uniprot.org\/uniprotkb?query=FXN - NCBI Gene search — NCBI — https:\/\/www.ncbi.nlm.nih.gov\/gene\/?term=FXN - Ensembl search — Ensembl — https:\/\/www.ensembl.org\/Multi\/Search\/Results?q=FXN - Human Protein Atlas search — HPA — https:\/\/www.proteinatlas.org\/search\/FXN - PubMed (review) — NLM — https:\/\/pubmed.ncbi.nlm.nih.gov\/?term=FXN+review --\u003e","brand":"NSJ Bioreagents","offers":[{"title":"Adding 0.2 ml of distilled water will yield a concentration of 500 ug\/ml \/ 100 ug","offer_id":53047322771821,"sku":"FY13347","price":449.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0949\/7424\/7277\/files\/get_image_8646df8b-623c-4fa6-8010-a821d575e1f6.jpg?v=1782237104","url":"https:\/\/www.ebiohippo.com\/products\/fxn-antibody-frataxin-bha17136249","provider":"BioHippo","version":"1.0","type":"link"}