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- bs-9601R
- 2025年10月16日
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- 规格：
50ul/100ul/200ul
| 规格： | 50ul | 产品价格： | ￥1180.0 |
|---|---|---|---|
| 规格： | 100ul | 产品价格： | ￥1980.0 |
| 规格： | 200ul | 产品价格： | ￥2800.0 |
| 产品编号 | bs-9601R |
| 英文名称 | Frataxin Rabbit pAb |
| 中文名称 | 线粒体型共济失调蛋白抗体 |
| 英文别名 | X25; CyaY; d-FXN; FA antibody FARR; Frataxin mature form; Frataxin(81-210); FRDA; FRDA_HUMAN; Friedreich ataxia protein; FXN; i-FXN; m56-FXN; m78-FXN; m81-FXN; MGC57199; MSF01; MSF31; MSF42. |
| 产品应用 | WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 Not yet tested in other applications. |
| 交叉反应 | Human (Mouse, Rat, Chicken, Dog, Pig, Cow, Horse) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human Frataxin |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 19 kDa |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers Signal Transduction > Metabolism > Mitochondrial Tags & Cell Markers > Subcellular Markers > Organelles > Mitochondria |
| 亚基 | Belongs to the frataxin family. |
| 亚细胞定位 | Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. |
| 组织特异性 | Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. |
| 翻译后修饰 | Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure. |
| 相似性 | Belongs to the frataxin family. |
| 功能 | 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. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:100-500} |
Western blot analysis of FXN (arrow) using rabbit polyclonal FXN Antibody (C-term) . 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the FXN gene (Lane 2) .
Paraformaldehyde-fixed, paraffin embedded (human lung carcinoma); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (Frataxin ) Polyclonal Antibody, Unconjugated (bs-9601R) at 1:200 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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Frataxin Rabbit pAb(bs-9601R)-50ul/100ul/200ul
￥1180 - 2800
