| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | SPG7 |
| Uniprot No | Q9UQ90 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-795 aa |
| 活性数据 | MAVLLLLLRALRRGPGPGPRPLWGPGPAWSPGFPARPGRGRPYMASRPPGDLAEAGGRALQSLQLRLLTPTFEGINGLLLKQHLVQNPVRLWQLLGGTFYFNTSRLKQKNKEKDKSKGKAPEEDEEERRRRERDDQMYRERLRTLLVIAVVMSLLNALSTSGGSISWNDFVHEMLAKGEVQRVQVVPESDVVEVYLHPGAVVFGRPRLALMYRMQVANIDKFEEKLRAAEDELNIEAKDRIPVSYKRTGFFGNALYSVGMTAVGLAILWYVFRLAGMTGREGGFSAFNQLKMARFTIVDGKMGKGVSFKDVAGMHEAKLEVREFVDYLKSPERFLQLGAKVPKGALLLGPPGCGKTLLAKAVATEAQVPFLAMAGPEFVEVIGGLGAARVRSLFKEARARAPCIVYIDEIDAVGKKRSTTMSGFSNTEEEQTLNQLLVEMDGMGTTDHVIVLASTNRADILDGALMRPGRLDRHVFIDLPTLQERREIFEQHLKSLKLTQSSTFYSQRLAELTPGFSGADIANICNEAALHAAREGHTSVHTLNFEYAVERVLAGTAKKSKILSKEEQKVVAFHESGHALVGWMLEHTEAVMKVSITPRTNAALGFAQMLPRDQHLFTKEQLFERMCMALGGRASEALSFNEVTSGAQDDLRKVTRIAYSMVKQFGMAPGIGPISFPEAQEGLMGIGRRPFSQGLQQMMDHEARLLVAKAYRHTEKVLQDNLDKLQALANALLEKEVINYEDIEALIGPPPHGPKKMIAPQRWIDAQREKQDLGEEETEETQQPPLGGEEPTWPK |
| 分子量 | 114.6 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 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. |
以下是关于重组人SPG7蛋白的参考文献示例，基于现有研究领域推测，实际引用时建议通过数据库核对详细信息：
1. **《Mutations in the mitochondrial protease gene SPG7 cause hereditary spastic paraplegia》**
- 作者：Casari, G. 等
- 摘要：报道SPG7基因突变导致遗传性痉挛性截瘫，通过重组SPG7蛋白证实其作为线粒体金属蛋白酶的功能，突变体酶活性降低导致轴突退化。
2. **《Structural and functional characterization of the human mitochondrial protease SPG7》**
- 作者：Hansen, J. 等
- 摘要：利用大肠杆菌重组表达SPG7蛋白，解析其三维结构，揭示其底物结合域及致病突变对蛋白水解活性的影响。
3. **《SPG7 interacts with PARL to promote mitochondrial proteostasis》**
- 作者：Klebe, S. 等
- 摘要：通过重组SPG7与PARL共表达实验，证明两者形成复合物，协同调控线粒体内膜蛋白质量控制，缺失导致神经退行性病变。
4. **《Recombinant SPG7 delivery rescues mitochondrial dysfunction in HSP models》**
- 作者：Piro-Mégy, C. 等
- 摘要：在SPG7缺陷小鼠模型中，注射重组SPG7蛋白可恢复线粒体膜电位并改善运动功能障碍，提示其治疗潜力。
**注意**：以上为模拟示例，实际文献需以PubMed/Google Scholar为准，建议结合关键词“recombinant SPG7 protein”或“SPG7 purification”检索最新研究。
Recombinant human SPG7 protein is derived from the SPG7 gene, which encodes paraplegin, a mitochondrial metalloprotease crucial for maintaining mitochondrial function. Mutations in SPG7 are linked to hereditary spastic paraplegia type 7 (HSP7), a neurodegenerative disorder characterized by progressive lower-limb spasticity, muscle weakness, and occasionally optic atrophy or cerebellar ataxia. Paraplegin, localized to the mitochondrial inner membrane, forms a heteromeric complex with AFG3L2. participating in protein quality control, ribosomal assembly, and the regulation of mitochondrial dynamics. It plays a vital role in cleaving misfolded proteins, ensuring proper mitochondrial membrane potential, and sustaining axonal integrity in long spinal cord neurons.
The production of recombinant SPG7 protein enables functional studies to dissect molecular mechanisms underlying HSP7. Researchers use in vitro models to investigate how SPG7 mutations impair proteolytic activity, disrupt mitochondrial homeostasis, or induce oxidative stress, contributing to neuronal degeneration. Additionally, recombinant SPG7 serves as a tool for drug screening, aiming to identify compounds that restore mitochondrial function or compensate for enzymatic deficiencies. Its applications extend to structural studies, epitope mapping for antibody development, and potential therapeutic strategies like gene therapy or enzyme replacement. Standard purification methods involve expression in eukaryotic systems (e.g., HEK293 cells) to ensure proper post-translational modifications and functional fidelity.
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