Frataxin Antibody / FXN

• Catalog Number: orb2641508
• Category: Antibodies
• Description: Reduced expression of frataxin is the cause of Friedreich's ataxia (FRDA), a lethal neurodegenerative disease. The reduction in frataxin gene expression may be attributable from either the silencing of transcription of the frataxin gene because of epigenetic modifications in the chromosomal entity or from the inability of splicing the expanded GAA repeats in the first intron of the pre-mRNA as seen in bacteria and human cells or both. The expansion of intronic trinucleotide repeat GAA results in Friedreich's ataxia. [Wiki]
• Species/Host: Mouse
• Clonality: Monoclonal
• Clone Number: FDAX-1
• Tested applications: IHC-P, WB
• Reactivity: Human
• Isotype: Mouse IgG2b, kappa
• Immunogen: Amino acids 57-210 from the human protein were used as the immunogen for this Frataxin antibody.
• Antibody Type: Primary Antibody
• Dilution range: Western blot: 1-2ug/ml,Immunohistochemistry (FFPE): 1-2ug/ml for 30 min at RT,Prediluted IHC only format: incubate for 30 min at RT (1)
• Purity: Protein G affinity chromatography
• Conjugation: Unconjugated
• Formula: 0.2 mg/ml in 1X PBS with 0.1 mg/ml BSA (US sourced) and 0.05% sodium azide
• Hazard Information: This Frataxin antibody is available for research use only.
• UniProt ID: Q16595
• Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
• Buffer/Preservatives: 0.2 mg/ml in 1X PBS with 0.1 mg/ml rAlbumin (US sourced) and 0.05% sodium azide
• Note: For research use only
• Application notes: Optimal dilution of the Frataxin antibody should be determined by the researcher.1. The prediluted format is supplied in a dropper bottle and is optimized for use in IHC. After epitope retrieval step (if required), drip mAb solution onto the tissue section and incubate at RT for 30 min.
• Expiration Date: 12 months from date of receipt.

Western blot testing of Fascin antibody and human HepG2 cell lysate (clone FDAX-1). Expected molecular weight: ~23 kDa (full length), ~14 kDa (mature).

IHC testing of FFPE human pancreas with Frataxin antibody (clone FDAX-1). Required HIER: boil tissue sections in pH9 10mM Tris with 1mM EDTA for 10-20 min and allow to cool before testing.