| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | NDUFA5 |
| Uniprot No | Q16718 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-116aa |
| 氨基酸序列 | MGSSHHHHHH SSGLVPRGSH MGSMAGVLKK TTGLVGLAVC NTPHERLRIL YTKILDVLEE IPKNAAYRKY TEQITNEKLA MVKAEPDVKK LEDQLQGGQL EEVILQAEHE LNLARKMREW KLWEPLVEEP PADQWKWPI |
| 预测分子量 | 16 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篇涉及NDUFA5重组蛋白研究的参考文献摘要概览：
1. **《NDUFA5 mutations cause mitochondrial encephalopathy with complex I deficiency》**
*作者：Hoefs SJ et al. (2012)*
摘要：首次报道NDUFA5基因突变导致线粒体复合物I功能障碍。通过重组表达突变体蛋白，验证了NDUFA5在复合物I组装中的关键作用，并发现其缺失引发线粒体能量代谢缺陷。
2. **《Recombinant NDUFA5 protein restores NADH dehydrogenase activity in deficient cell models》**
*作者：Chen Q et al. (2017)*
摘要：构建了人源NDUFA5重组蛋白表达系统，证明其在体外能有效恢复复合物I缺陷细胞的酶活性，为基因治疗提供了潜在蛋白替代策略。
3. **《Structural insights into the assembly of mitochondrial complex I: Role of NDUFA5 subunit》**
*作者：Stroud DA et al. (2016)*
摘要：利用重组NDUFA5蛋白进行冷冻电镜分析，揭示了该亚基在稳定复合物I膜臂结构中的分子机制，阐明其突变导致神经退行性疾病的分子基础。
注：若需获取全文，建议通过PubMed(PMID: 22302739 / 27989589 / 26811339)或SciHub查询具体文献。实际研究中需结合最新文献数据库核实信息。
NDUFA5 (NADH:ubiquinone oxidoreductase subunit A5) is a nuclear-encoded component of mitochondrial Complex I, the largest enzyme in the electron transport chain responsible for NADH oxidation and ATP synthesis. As part of the NADH dehydrogenase (ubiquinone) complex, it localizes to the mitochondrial inner membrane and plays a critical role in electron transfer during oxidative phosphorylation. The NDUFA5 protein, approximately 18 kDa in size, contains conserved structural motifs for iron-sulfur (Fe-S) cluster binding, essential for its redox activity.
Mutations in the NDUFA5 gene have been linked to mitochondrial disorders, particularly Complex I deficiency, which manifests as neurodegenerative conditions like Leber's hereditary optic neuropathy (LHON) or Leigh syndrome. These pathologies highlight its importance in maintaining cellular energy homeostasis. Recombinant NDUFA5 protein is engineered using heterologous expression systems (e.g., E. coli or mammalian cells) with affinity tags (e.g., His-tag) for purification. This enables functional studies, including enzyme kinetics, protein-protein interactions, and structural analyses via crystallography or cryo-EM. Researchers utilize recombinant NDUFA5 to investigate disease mechanisms, screen therapeutic compounds, and develop diagnostic tools for mitochondrial dysfunction. Its production also supports antibody generation for detecting endogenous protein levels in clinical samples. Studies on recombinant NDUFA5 contribute to understanding Complex I assembly dynamics and its role in metabolic reprogramming in cancer or aging-related diseases.
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