验证数据展示
经过测试的应用
| Positive WB detected in | HCT 116 cells, Jurkat cells, K-562 cells, Raji cells, mouse liver tissue |
| Positive IHC detected in | human stomach tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Western Blot (WB) | WB : 1:1000-1:6000 |
| Immunohistochemistry (IHC) | IHC : 1:300-1:1200 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
产品信息
14147-1-AP targets Frataxin in WB, IHC, IF, CoIP, ELISA applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | WB, IHC, ELISA Application Description |
| 文献引用应用 | WB, IHC, IF, CoIP |
| 经测试反应性 | human, mouse, rat |
| 文献引用反应性 | human, mouse, rat |
| 免疫原 | Frataxin fusion protein Ag5316 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | frataxin |
| 别名 | FXN, FA, Extramitochondrial frataxin, EC:1.16.3.1, dFXN |
| 计算分子量 | 23 kDa |
| 观测分子量 | 14 kDa |
| GenBank蛋白编号 | BC048097 |
| 基因名称 | FXN |
| Gene ID (NCBI) | 2395 |
| RRID | AB_2231876 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | Q16595 |
| 储存缓冲液 | PBS with 0.02% sodium azide and 50% glycerol , pH 7.3 |
| 储存条件 | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
Frataxin (FXN), also named as FRDA, X25, m81-FXN, d-FXN, m78-FXN and i-FXN, belongs to the frataxin family. It promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. FXN may play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. FXN is cleaved to be 4 chains. FXN is expressed in the cytoplasm as a 210 amino acid (AA) precursor protein (pFXN; 23 KDa) that is translocated into mitochondria where it is processed by two consecutive steps into iFXN (FXN 42-210; 19 KDa) and finally mFXN (81-210; 14.2 KDa), which is functional. (PMID: 26704351, PMID: 26671574)
实验方案
| Product Specific Protocols | |
|---|---|
| WB protocol for Frataxin antibody 14147-1-AP | Download protocol |
| IHC protocol for Frataxin antibody 14147-1-AP | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
发表文章
| Species | Application | Title |
|---|---|---|
Blood Defective palmitoylation of transferrin receptor triggers iron overload in Friedreich ataxia fibroblasts. | ||
Neurobiol Dis Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin | ||
J Nutr Biochem Iron-frataxin involved in the protective effect of quercetin against alcohol-induced liver mitochondrial dysfunction | ||
Sci Rep Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich's ataxia. | ||
Hum Mol Genet Premature transcription termination at the expanded GAA repeats and aberrant alternative polyadenylation contributes to the Frataxin transcriptional deficit in Friedreich's ataxia. |