| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | ELAC2 |
| Uniprot No | Q9BQ52 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-826aa |
| 氨基酸序列 | MWALCSLLRSAAGRTMSQGRTISQAPARRERPRKDPLRHLRTREKRGPSGCSGGPNTVYLQVVAAGSRDSGAALYVFSEFNRYLFNCGEGVQRLMQEHKLKVARLDNIFLTRMHWSNVGGLSGMILTLKETGLPKCVLSGPPQLEKYLEAIKIFSGPLKGIELAVRPHSAPEYEDETMTVYQIPIHSEQRRGKHQPWQSPERPLSRLSPERSSDSELNENEPHLPHGVSQRRGVRDSSLVVAFICKLHLKRGNFLVLKAKEMGLPVGTAAIAPIIAAVKDGKSITHEGREILAEELCTPPDPGAAFVVVECPDESFIQPICENATFQRYQGKADAPVALVVHMAPASVLVDSRYQQWMERFGPDTQHLVLNENCASVHNLRSHKIQTQLNLIHPDIFPLLTSFRCKKEGPTLSVPMVQGECLLKYQLRPRREWQRDAIITCNPEEFIVEALQLPNFQQSVQEYRRSAQDGPAPAEKRSQYPEIIFLGTGSAIPMKIRNVSATLVNISPDTSLLLDCGEGTFGQLCRHYGDQVDRVLGTLAAVFVSHLHADHHTGLPSILLQRERALASLGKPLHPLLVVAPNQLKAWLQQYHNQCQEVLHHISMIPAKCLQEGAEISSPAVERLISSLLRTCDLEEFQTCLVRHCKHAFGCALVHTSGWKVVYSGDTMPCEALVRMGKDATLLIHEATLEDGLEEEAVEKTHSTTSQAISVGMRMNAEFIMLNHFSQRYAKVPLFSPNFSEKVGVAFDHMKVCFGDFPTMPKLIPPLKALFAGDIEEMEERREKRELRQVRAALLSRELAGGLEDGEPQQKRAHTEEPQAKKVRAQ |
| 分子量 | 118.6 kDa |
| 蛋白标签 | GST-tag at N-terminal |
| 缓冲液 | 0 |
| 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. |
1. **"Structural and Functional Analysis of Human ELAC2" by Shutt, T.E., et al.**
- 摘要：该研究解析了重组人ELAC2蛋白的晶体结构，揭示了其作为线粒体tRNA成熟关键核酸酶的活性位点构象，并证实其催化锌离子依赖性水解反应的机制。
2. **"ELAC2 Mutations Cause Mitochondrial RNA Processing Defects" by Haack, T.B., et al.**
- 摘要：通过重组蛋白表达与功能实验，证明ELAC2基因突变导致线粒体tRNA 3'端加工异常，并与婴儿期线粒体脑肌病相关，阐明了其病理机制。
3. **"Biochemical Characterization of Recombinant ELAC2 Protein" by Tavtigian, S.V., et al.**
- 摘要：利用大肠杆菌表达系统纯化重组ELAC2蛋白，验证其在体外对前体tRNA的切割活性，并发现部分前列腺癌相关突变会显著降低其酶活性。
4. **"Functional Interaction of ELAC2 with Mitochondrial Translation Machinery" by Tucci, S., et al.**
- 摘要：研究重组ELAC2与线粒体核糖体蛋白的相互作用，表明其通过调控tRNA成熟影响线粒体翻译效率，可能参与能量代谢疾病的分子通路。
注：以上文献名为虚拟示例，实际研究中需查阅具体数据库(如PubMed)获取真实文献。
Recombinant human ELAC2 protein is a genetically engineered version of the endonuclease encoded by the ELAC2 gene, which plays a critical role in mitochondrial RNA processing. ELAC2 is a zinc-dependent metallophosphatase responsible for the cleavage of 5' leader sequences during the maturation of transfer RNA (tRNA) precursors in mitochondria. This enzymatic activity is essential for mitochondrial protein synthesis and, consequently, mitochondrial function. Mutations in the ELAC2 gene have been linked to severe human disorders, including infantile-onset hypertrophic cardiomyopathy, mitochondrial encephalopathy, and hereditary prostate cancer, highlighting its biological and clinical significance.
The recombinant protein is typically produced in heterologous expression systems such as *E. coli* or mammalian cell cultures, enabling large-scale purification for functional studies. Researchers utilize recombinant ELAC2 to investigate its structural and enzymatic properties, including substrate specificity, catalytic mechanisms, and interactions with other mitochondrial proteins. Its study also provides insights into the molecular basis of mitochondrial tRNA processing defects and associated pathologies. Additionally, recombinant ELAC2 serves as a tool for screening potential therapeutic compounds targeting mitochondrial dysfunction or prostate cancer. Understanding ELAC2's role in disease pathways may pave the way for novel diagnostics or therapies addressing mitochondrial disorders or malignancies linked to its dysregulation.
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