| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | TPP1 |
| Uniprot No | O14773 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 196-563aa |
| 氨基酸序列 | LHLGV TPSVIRKRYN LTSQDVGSGT SNNSQACAQF LEQYFHDSDL AQFMRLFGGN FAHQASVARV VGQQGRGRAG IEASLDVQYL MSAGANISTW VYSSPGRHEG QEPFLQWLML LSNESALPHV HTVSYGDDED SLSSAYIQRV NTELMKAAAR GLTLLFASGD SGAGCWSVSG RHQFRPTFPA SSPYVTTVGG TSFQEPFLIT NEIVDYISGG GFSNVFPRPS YQEEAVTKFL SSSPHLPPSS YFNASGRAYP DVAALSDGYW VVSNRVPIPW VSGTSASTPV FGGILSLINE HRILSGRPPL GFLNPRLYQQ HGAGLFDVTR GCHESCLDEE VEGQGFCSGP GWDPVTGWGT PNFPALLKTL LNP |
| 预测分子量 | 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篇关于TPP1重组蛋白的文献示例(注：部分信息为模拟概括，实际文献需核实)：
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1. **文献名称**：*Structural basis of TPP1 recruitment to telomerase through a conserved peptide interaction*
**作者**：Nandakumar, J., et al.
**摘要**：解析了TPP1重组蛋白的OB结构域与端粒酶RNA亚基的相互作用机制，通过X射线晶体学揭示其结合位点，阐明TPP1在端粒酶复合体组装中的关键作用。
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2. **文献名称**：*Recombinant TPP1 restores telomere length in dyskeratosis congenita cells*
**作者**：Sexton, A.N., et al.
**摘要**：研究重组TPP1蛋白在先天性角化不良症患者细胞模型中的应用，发现其通过激活端粒酶活性修复端粒缩短，为基因治疗提供实验依据。
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3. **文献名称**：*TPP1-POT1 heterodimer coordinates telomere protection and elongation*
**作者**：Zhong, F.L., et al.
**摘要**：利用重组TPP1/POT1复合体进行体外实验，证明其协同保护端粒DNA免受降解，并通过调控端粒酶活性促进端粒延长。
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4. **文献名称**：*Engineering of recombinant TPP1 for enhanced telomerase processivity*
**作者**：Schmidt, J.C., et al.
**摘要**：通过蛋白质工程优化重组TPP1结构，提高其与端粒酶的亲和力，显著增强端粒酶在体外延伸端粒的持续合成能力。
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**注**：以上文献名称及内容为示例性质，具体研究请以实际发表的论文为准。建议通过PubMed或Web of Science检索关键词“TPP1 recombinant protein”获取真实文献。
TPP1 (tripeptidyl peptidase 1), also known as lysosomal acid serine protease, is a key enzyme involved in lysosomal protein degradation. As a member of the serine-carboxypeptidase family, it sequentially removes tripeptides from the N-terminus of substrate proteins, playing a critical role in maintaining cellular homeostasis. Naturally synthesized as an inactive proenzyme, TPP1 undergoes proteolytic activation within lysosomes to become a functional protease.
The clinical significance of TPP1 emerged through its association with neuronal ceroid lipofuscinosis type 2 (CLN2 disease), a fatal pediatric neurodegenerative disorder caused by TPP1 deficiency. This autosomal recessive condition results in lysosomal accumulation of undigested material, leading to progressive neurological deterioration. This connection spurred research into recombinant TPP1 as a therapeutic intervention.
Recombinant TPP1 is typically produced using mammalian expression systems (e.g., CHO cells) to ensure proper post-translational modifications and enzymatic activity. The purification process involves affinity chromatography and quality verification through activity assays and structural characterization. Pharmaceutical-grade recombinant TPP1 (e.g., cerliponase alfa) has been developed as enzyme replacement therapy (ERT), administered via intracerebroventricular infusion to bypass the blood-brain barrier.
Current applications extend beyond CLN2 treatment, including its use as a research tool for studying lysosomal biology and protein catabolism. Challenges persist in optimizing delivery methods, enhancing enzyme stability, and managing immune responses. Ongoing clinical trials continue to evaluate long-term efficacy and safety profiles, with approved therapies demonstrating measurable delays in disease progression. The development of TPP1 biologics represents a milestone in treating lysosomal storage disorders while highlighting the complexities of targeting neurological diseases.
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