| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | TSEN54 |
| Uniprot No | Q7Z6J9 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-526 aa |
| 活性数据 | MEPEPEPAAV EVPAGRVLSA RELFAARSRS QKLPQRSHGP KDFLPDGSAA QAERLRRCRE ELWQLLAEQR VERLGSLVAA EWRPEEGFVE LKSPAGKFWQ TMGFSEQGRQ RLHPEEALYL LECGSIHLFH QDLPLSIQEA YQLLLTDHTV TFLQYQVFSH LKRLGYVVRR FQPSSVLSPY ERQLNLDASV QHLEDGDGKR KRSSSSPRSI NKKAKALDNS LQPKSLAASS PPPCSQPSQC PEEKPQESSP MKGPGGPFQL LGSLGPSPGP AREGVGCSWE SGRAENGVTG AGKRRWNFEQ ISFPNMASDS RHTLLRAPAP ELLPANVAGR ETDAESWCQK LNQRKEKLSR REREHHAEAA QFQEDVNADP EVQRCSSWRE YKELLQRRQV QRSQRRAPHL WGQPVTPLLS PGQASSPAVV LQHISVLQTT HLPDGGARLL EKSGGLEIIF DVYQADAVAT FRKNNPGKPY ARMCISGFDE PVPDLCSLKR LSYQSGDVPL IFALVDHGDI SFYSFRDFTL PQDVGH |
| 分子量 | 58.8 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. |
以下是关于重组人TSEN54蛋白的3篇参考文献示例，内容基于相关研究领域，仅供参考：
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1. **标题**：*Structural insights into the human tRNA splicing endonuclease complex*
**作者**：J. Smith et al.
**摘要**：研究通过重组表达人TSEN54、TSEN2、TSEN34和TSEN15亚基，成功解析了TSEN复合物的三维结构，揭示了TSEN54在底物识别和催化中的关键作用，为理解其tRNA剪切机制提供依据。
2. **标题**：*Functional characterization of TSEN54 mutations in pontocerebellar hypoplasia*
**作者**：L. Müller et al.
**摘要**：通过重组TSEN54蛋白及其突变体的体外实验，发现某些突变会破坏TSEN复合物的稳定性并降低tRNA剪切活性，解释了这些突变如何导致小脑发育异常的分子机制。
3. **标题**：*Recombinant expression and biochemical analysis of the human TSEN54 protein*
**作者**：K. Brown et al.
**摘要**：描述了在HEK293细胞中高效表达和纯化重组人TSEN54蛋白的优化方法，并验证其与其他TSEN亚基的相互作用，为后续功能研究提供技术基础。
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**注**：以上文献为示例性内容，实际研究中建议通过PubMed或Google Scholar以关键词“recombinant TSEN54”或“TSEN complex structure”检索最新论文。
**Background of Recombinant Human TSEN54 Protein**
TSEN54 (tRNA splicing endonuclease subunit 54) is a critical component of the tRNA splicing endonuclease complex, which is essential for processing precursor tRNAs (pre-tRNAs) by removing introns during tRNA maturation. This protein, encoded by the *TSEN54* gene in humans, works alongside other subunits (TSEN2. TSEN15. and TSEN34) to ensure precise cleavage at the splice junctions, a step vital for generating functional tRNAs and maintaining translational accuracy.
Mutations in *TSEN54* are strongly linked to pontocerebellar hypoplasia (PCH), a group of severe neurodevelopmental disorders. Specifically, PCH types 2. 4. and 5 are associated with autosomal recessive *TSEN54* variants, leading to brainstem and cerebellar atrophy, microcephaly, and early mortality. These findings highlight TSEN54's role in neural development and cellular homeostasis.
Recombinant human TSEN54 protein is engineered using expression systems (e.g., *E. coli* or mammalian cells) to produce purified, functional protein for research. It enables studies on tRNA splicing mechanisms, disease-related mutations, and interactions within the endonuclease complex. Additionally, recombinant TSEN54 aids in structural analyses (e.g., X-ray crystallography) and high-throughput screening for therapeutic strategies targeting PCH. Understanding TSEN54's molecular functions and dysregulation provides insights into tRNA processing disorders and potential interventions.
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