| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | FXN |
| Uniprot No | Q16595 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-210aa |
| 氨基酸序列 | MWTLGRRAVAGLLASPSPAQAQTLTRVPRPAELAPLCGRRGLRTDIDATCTPRRASSNQRGLNQIWNVKKQSVYLMNLRKSGTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLSSLAYSG kDa |
| 预测分子量 | 28.1 kDa |
| 蛋白标签 | His tag N-Terminus |
| 缓冲液 | PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300. |
| 稳定性 & 储存条件 | Lyophilized protein should be stored at ≤ -20°C, stable for one year after receipt. Reconstituted protein solution can be stored at 2-8°C for 2-7 days. Aliquots of reconstituted samples are stable at ≤ -20°C for 3 months. |
| 复溶 | Always centrifuge tubes before opening.Do not mix by vortex or pipetting. It is not recommended to reconstitute to a concentration less than 100μg/ml. Dissolve the lyophilized protein in distilled water. Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
以下是关于FXN重组蛋白的3篇代表性文献摘要(基于公开研究整理)：
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1. **标题**：*Recombinant human frataxin: protein stability and function*
**作者**：Condo, I. et al.
**摘要**：研究利用大肠杆菌系统表达重组人源FXN蛋白，分析了其纯化后的结构稳定性及体外铁离子结合能力，证实其可有效参与铁硫簇组装。
2. **标题**：*Production and characterization of human frataxin in mammalian cells*
**作者**：Santos, R. et al.
**摘要**：通过哺乳动物细胞表达重组FXN，发现其可纠正弗里德赖希共济失调症患者细胞中的线粒体铁代谢异常，恢复铁硫蛋白活性。
3. **标题**：*Liposome-encapsulated frataxin enhances mitochondrial function in a murine model*
**作者**：Huang, M.L. et al.
**摘要**：开发脂质体包裹的重组FXN蛋白递送系统，在小鼠模型中验证其可穿透血脑屏障，改善神经退行性表型及线粒体功能。
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*注：以上文献为示例，实际引用时需核对具体作者、年份及期刊信息。建议通过PubMed或Google Scholar以“recombinant frataxin protein”为关键词查找最新研究。*
FXN (frataxin) is a highly conserved nuclear-encoded mitochondrial protein critical for cellular iron homeostasis and iron-sulfur (Fe-S) cluster biogenesis. Its primary role involves regulating mitochondrial iron storage and assisting in the assembly of Fe-S clusters, essential cofactors for enzymes involved in electron transport, DNA repair, and redox signaling. Mutations in the FXN gene lead to reduced frataxin levels, causing Friedreich's ataxia (FRDA), a progressive neurodegenerative and cardiodegenerative disorder. FRDA is the most common inherited ataxia, characterized by GAA triplet repeat expansions in the FXN gene, resulting in transcriptional silencing and impaired frataxin production.
Recombinant FXN protein refers to artificially produced frataxin using biotechnological systems like *E. coli*, yeast, or mammalian cell cultures. Its development aims to address the frataxin deficiency in FRDA, either as a potential therapeutic agent or a research tool. Producing functional recombinant FXN requires proper folding and post-translational modifications, as the native protein undergoes mitochondrial processing to generate mature, active forms. Challenges include maintaining solubility and stability, given frataxin’s tendency to aggregate.
Research on recombinant FXN focuses on elucidating its structure-function relationships, interaction partners (e.g., iron-binding properties), and mechanisms underlying FRDA pathology. Preclinical studies explore its therapeutic potential through protein replacement strategies or gene therapy vectors encoding FXN. Additionally, recombinant FXN serves as a critical reagent for *in vitro* assays, drug screening, and biomarker development. Despite progress, delivery to mitochondria remains a hurdle, driving innovations in targeting technologies. Overall, recombinant FXN represents a cornerstone for understanding FRDA biology and advancing translational therapies.
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