FITC标记的线粒体型共济失调蛋白抗体
产品名称： FITC标记的线粒体型共济失调蛋白抗体
英文名称： Anti-Frataxin/FITC
产品编号： HZ-9601R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-Frataxin/FITC Conjugated antibody
FITC标记的线粒体型共济失调蛋白抗体
| 产品编号 | bs-9601R-FITC |
| 英文名称 | Anti-Frataxin/FITC |
| 中文名称 | FITC标记的线粒体型共济失调蛋白抗体 |
| 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. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 心血管 细胞生物 免疫学 神经生物学 信号转导 线粒体 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 19kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Frataxin |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | 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. 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. Subunit: Belongs to the frataxin family. Subcellular Location: Cytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria. Tissue Specificity: Expressed in the heart, peripheral blood lymphocytes and dermal fibroblasts. Post-translational modifications: 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. DISEASE: Defects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region. [MISCELLANEOUS] The unusual migration profile of mature frataxin on SDS-PAGE due to its acidic N-terminus most likely contributed to conflicting reports for the N-terminus of the mature protein. Unlike prokaryotic and yeast frataxin homologs, which self-assemble at high iron concentrations, oligomerization of human frataxin is not induced by iron. The existence of a specialized mitochondrial ferritin in mammalia (FTMT) is suggesting that iron storage would be redundant function, at least in mammalian mitochondria. Similarity: Belongs to the frataxin family. Database links: Entrez Gene: 2395 Human Omim: 606829 Human SwissProt: Q16595 Human Unigene: 20685 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
Friedreich ataxia是由Frasxin基因功能丧失突变引起的渐进性神经退行性疾病。人Frasxin基因定位于染色体9q13，Frasxin基因编码一个同名的线粒体蛋白。Frasxin组装成具有铁结合能力的稳定均聚物。当在大肠杆菌中表达时，人Frasxin以每1个Frasxin聚合物分子的10个铁原子的速率结合铁原子。因此，Frasxin似乎在一定程度上起着线粒体铁储存的作用。Frasxin也可作为氧化磷酸化的活化剂，以增加线粒体膜电位并升高细胞ATP。Frasxin在具有高代谢活性的组织中表达，包括心脏、肝脏和棕色脂肪。