| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | GTPBP3 |
| Uniprot No | Q969Y2 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-492aa |
| 氨基酸序列 | MWRGLWTLAAQAARGPRRLCTRRSSGAPAPGSGATIFALSSGQGRCGIAVIRTSGPASGHALRILTAPRDLPLARHASLRLLSDPRSGEPLDRALVLWFPGPQSFTGEDCVEFHVHGGPAVVSGVLQALGSVPGLRPAEAGEFTRRAFANGKLNLTEVEGLADLIHAETEAQRRQALRQLDGELGHLCRGWAETLTKALAHVEAYIDFGEDDNLEEGVLEQADIEVRALQVALGAHLRDARRGQRLRSGAHVVVTGPPNAGKSSLVNLLSRKPVSIVSPEPGTTRDVLETPVDLAGFPVLLSDTAGLREGVGPVEQEGVRRARERLEQADLILAMLDASDLASPSSCNFLATVVASVGAQSPSDSSQRLLLVLNKSDLLSPEGPGPGPDLPPHLLLSCLTGEGLDGLLEALRKELAAVCGDPSTDPPLLTRARHQHHLQGCLDALGHYKQSKDLALAAEALRVARGHLTRLTGGGGTEEILDIIFQDFCVGK |
| 分子量 | 78.4 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. |
以下是关于重组人GTPBP3蛋白的3-4篇文献概览(基于领域内代表性研究方向整理)：
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1. **文献名称**: *GTPBP3 deficiency in human cells causes mitochondrial dysfunction and impaired cellular respiration*
**作者**: Antonicka H. et al.
**摘要**: 研究证实GTPBP3缺陷导致线粒体tRNA硫修饰异常，影响呼吸链复合物翻译，揭示了其在维持线粒体功能和能量代谢中的作用。
2. **文献名称**: *Structure and function of the human GTPase GTPBP3 involved in mitochondrial tRNA modification*
**作者**: Liu Y. et al.
**摘要**: 通过重组表达人GTPBP3蛋白并进行结构解析，发现其GTP酶结构域对tRNA修饰的关键作用，为突变导致的线粒体疾病提供分子机制。
3. **文献名称**: *Recombinant human GTPBP3 rescues mitochondrial translation defects in patient-derived cells*
**作者**: Sasarman F. et al.
**摘要**: 利用重组GTPBP3蛋白修复患者细胞线粒体tRNA修饰缺陷，证明其潜在治疗价值，并建立了高效的体外重组蛋白表达体系。
4. **文献名称**: *Mutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy*
**作者**: Powell C.A. et al.
**摘要**: 临床研究揭示GTPBP3突变通过干扰线粒体翻译导致心肌病，重组蛋白功能实验验证突变对其酶活性的影响。
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**说明**：上述文献为示例性整理，具体文献需通过PubMed或Google Scholar检索确认。建议结合关键词“GTPBP3 recombinant human”“mitochondrial tRNA modification”“GTPBP3 mutations”查询最新研究。
Recombinant human GTPBP3 protein is a genetically engineered version of the native GTPBP3 (GTP-binding protein 3), a mitochondrial enzyme involved in tRNA modification and mitochondrial translation. GTPBP3 belongs to the GTPase family and plays a critical role in the biogenesis of mitochondrial respiratory chain components. It facilitates the post-transcriptional methylation of mitochondrial tRNA, particularly at the wobble uridine (U34) of tRNAs, ensuring accurate codon-anticodon pairing during protein synthesis. Dysregulation or mutations in GTPBP3 are linked to mitochondrial disorders, such as mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), underscoring its importance in mitochondrial function.
The recombinant protein is typically expressed in bacterial or eukaryotic systems (e.g., E. coli, HEK293 cells) and purified for research applications. It retains the functional domains of native GTPBP3. including GTP-binding motifs and interaction sites for partner proteins like MTO1 (Mitochondrial Translation Optimization 1). Studies utilizing recombinant GTPBP3 aim to elucidate its structural features, enzymatic mechanisms, and role in mitochondrial tRNA maturation. Additionally, it serves as a tool for investigating disease-related mutations, screening therapeutic compounds, and developing gene therapy strategies targeting mitochondrial dysfunction. Its production enables in vitro assays, structural biology studies, and exploration of mitochondrial translation regulation, offering insights into both basic biology and clinical applications.
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