|Other Names||Frataxin, mitochondrial, Friedreich ataxia protein, Fxn, Frataxin intermediate form, i-FXN, Frataxin(56-210), m56-FXN, Frataxin(78-210), d-FXN, m78-FXN, Frataxin mature form, Frataxin(81-210), m81-FXN, FXN, FRDA, X25|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP6409b was selected from the C-term region of human FXN. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||The synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml deionized water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+); the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization; however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1.|
|Cellular Location||Mitochondrion Cytoplasm, cytosol Note=PubMed:18725397 reports localization exclusively in mitochondria.|
|Tissue Location||Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.|
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Provided below are standard protocols that you may find useful for product applications.
FXN is a mitochondrial protein which belongs to the FRATAXIN family. The protein functions in regulating mitochondrial iron transport and respiration. The expansion of intronic trinucleotide repeat GAA results in Friedreich ataxia.
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