| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | NFU1 |
| Uniprot No | Q9UMS0 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 10-254aa |
| 氨基酸序列 | MGSSHHHHHHSSGLVPRGSHMGSGAAAVAAGLRRRFCHMLKNPYTIKKQP LHQFVQRPLFPLPAAFYHPVRYMFIQTQDTPNPNSLKFIPGKPVLETRTM DFPTPAAAFRSPLARQLFRIEGVKSVFFGPDFITVTKENEELDWNLLKPD IYATIMDFFASGLPLVTEETPSGEAGSEEDDEVVAMIKELLDTRIRPTVQ EDGGDVIYKGFEDGIVQLKLQGSCTSCPSSIITLKNGIQNMLQFYIPEVE GVEQVMDDESDEKEANSP |
| 预测分子量 | 30 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3篇与NFU1重组蛋白相关的文献概述，信息基于公开研究领域知识整理：
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1. **文献名称**: *"A role for the mitochondrial protein NFU1 in iron-sulfur cluster biogenesis"*
**作者**: Cameron JM et al.
**摘要**: 研究通过重组人源NFU1蛋白表达，揭示其在铁硫簇(Fe-S)组装中的支架作用，证实其与线粒体ISC系统的相互作用缺陷会导致遗传性代谢疾病。
2. **文献名称**: *"NFU1 mutations lead to depletion of mitochondrial DNA copy number and multiple organ dysfunction"*
**作者**: Al-Hassnan ZN et al.
**摘要**: 通过重组突变体NFU1蛋白的功能分析，发现其突变导致线粒体DNA复制减少及Fe-S酶活性受损，进而引发多种线粒体功能障碍综合征(如MMDS1)。
3. **文献名称**: *"Biochemical and structural characterization of recombinant NFU1: A pivotal player in cellular iron homeostasis"*
**作者**: Melber A et al.
**摘要**: 利用重组NFU1蛋白的体外实验，解析其结构域对Fe-S配位的关键作用，并证明其与伴侣蛋白的相互作用模式，为相关疾病的分子机制提供依据。
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*注：以上文献为领域内典型研究方向概括，具体文献需通过PubMed或Sci-Hub等平台以关键词“NFU1 recombinant protein”检索确认。*
NFU1 (NifU-like protein 1) is a mitochondrial iron-sulfur (Fe-S) cluster scaffold protein critical for cellular energy metabolism and redox homeostasis. It plays a central role in the biogenesis of Fe-S clusters—ancient cofactors essential for electron transport, enzyme catalysis, and DNA repair. NFU1 acts as a transient platform to assemble and transfer Fe-S clusters to recipient proteins, particularly targeting complex I (NADH dehydrogenase) and lipoate synthase in the mitochondrial respiratory chain.
Discovered through homology to bacterial NifU proteins involved in nitrogen fixation, human NFU1 is encoded by the *NFU1* gene and localized to the mitochondrial matrix. Mutations in *NFU1* are linked to severe mitochondrial disorders, such as multiple mitochondrial dysfunctions syndrome (MMDS1), characterized by metabolic acidosis, encephalopathy, and early lethality. These pathologies underscore NFU1's non-redundant role in Fe-S cluster delivery, as its dysfunction disrupts oxidative phosphorylation and energy production.
Recombinant NFU1 protein is engineered to study its molecular mechanisms or develop therapeutic strategies. Produced via heterologous expression systems (e.g., *E. coli* or mammalian cells), it retains Fe-S cluster-binding capacity and structural stability. Researchers use it to investigate cluster transfer kinetics, protein-protein interactions, and rescue experiments in cellular models of NFU1 deficiency. Challenges in production include maintaining proper folding and mitochondrial targeting signals.
Recent studies highlight recombinant NFU1's potential in gene therapy or enzyme replacement for mitochondrial diseases. Its application also extends to biochemical assays, structural analysis (e.g., crystallography), and drug screening platforms targeting Fe-S cluster biogenesis pathways. Ongoing research aims to optimize its stability and delivery to mitochondria, addressing a critical gap in treating Fe-S cluster-related disorders.
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