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Anti-Frataxin Polyclonal Antibody is a Rabbit antibody targeting Frataxin. Anti-Frataxin Polyclonal Antibody can be used in WB,IHC-P,IHC-Fr,IF.
| Pack Size | Price | USA Warehouse | Global Warehouse | Quantity |
|---|---|---|---|---|
| 50 μL | $221 | 7-10 days | 7-10 days | |
| 100 μL | $374 | 7-10 days | 7-10 days | |
| 200 μL | $527 | 7-10 days | 7-10 days |
| Description | Anti-Frataxin Polyclonal Antibody is a Rabbit antibody targeting Frataxin. Anti-Frataxin Polyclonal Antibody can be used in WB,IHC-P,IHC-Fr,IF. |
| Ig Type | IgG |
| Reactivity | Human (predicted:Mouse,Rat,Chicken,Dog,Pig,Cow,Horse) |
| Application | |
| Recommended Dose | WB: 1:500-2000; IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500 |
| Antibody Type | Polyclonal |
| Host Species | Rabbit |
| Subcellular Localization | Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. |
| Tissue Specificity | Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. |
| Construction | Polyclonal Antibody |
| Purification | Protein A purified |
| Appearance | Liquid |
| Formulation | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Concentration | 1mg/ml |
| Research Background | Friedreich ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the frataxin gene. The human frataxin gene maps to chromosome 9q13.The frataxin gene encodes a mitochondrial protein of the same name. Frataxin assembles into a stable homopolymer with iron-binding capabilities. When expressed in E. Coli human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer. Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria. Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP. Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat. |
| Immunogen | KLH conjugated synthetic peptide: human Frataxin |
| Antigen Species | Human |
| Gene Name | FXN |
| Gene ID | |
| Protein Name | Frataxin, mitochondrial |
| Uniprot ID | |
| 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. |
| Molecular Weight | Theoretical: 19 kDa. |
| Stability & Storage | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| Transport | Shipping with blue ice. |
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