| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | RARS2 |
| Uniprot No | Q5T160 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 17-578aa |
| 氨基酸序列 | LNLP PENLITSISA VPISQKEEVA DFQLSVDSLL EKDNDHSRPD IQVQAKRLAE KLRCDTVVSE ISTGQRTVNF KINRELLTKT VLQQVIEDGS KYGLKSELFS GLPQKKIVVE FSSPNVAKKF HVGHLRSTII GNFIANLKEA LGHQVIRINY LGDWGMQFGL LGTGFQLFGY EEKLQSNPLQ HLFEVYVQVN KEAADDKSVA KAAQEFFQRL ELGDVQALSL WQKFRDLSIE EYIRVYKRLG VYFDEYSGES FYREKSQEVL KLLESKGLLL KTIKGTAVVD LSGNGDPSSI CTVMRSDGTS LYATRDLAAA IDRMDKYNFD TMIYVTDKGQ KKHFQQVFQM LKIMGYDWAE RCQHVPFGVV QGMKTRRGDV TFLEDVLNEI QLRMLQNMAS IKTTKELKNP QETAERVGLA ALIIQDFKGL LLSDYKFSWD RVFQSRGDTG VFLQYTHARL HSLEETFGCG YLNDFNTACL QEPQSVSILQ HLLRFDEVLY KSSQDFQPRH IVSYLLTLSH LAAVAHKTLQ IKDSPPEVAG ARLHLFKAVR SVLANGMKLL GITPVCRM |
| 预测分子量 | 65 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RARS2重组蛋白的3篇代表性文献及其摘要概括：
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1. **文献名称**：*Mutations in RARS2 cause pontocerebellar hypoplasia with infantile-onset parkinsonism*
**作者**：Edvardson S, et al.
**摘要概括**：
本研究首次将RARS2基因突变与早发性小脑萎缩和帕金森综合征相关联。作者通过重组蛋白表达验证了突变体(如p.Thr367Ile)的酶活性丧失，证明其损害线粒体tRNA精氨酸化功能，导致线粒体翻译缺陷。
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2. **文献名称**：*Functional analysis of RARS2 mutations linked to pontocerebellar hypoplasia reveals impaired mitochondrial translation*
**作者**：Li Z, et al.
**摘要概括**：
该研究在大肠杆菌中重组表达了野生型和突变型RARS2蛋白，通过体外酶活性和热稳定性实验，发现致病突变(如p.Leu152Val)显著降低精氨酰-tRNA合成酶活性，并破坏其结构稳定性，进而影响线粒体蛋白合成。
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3. **文献名称**：*Structural and biochemical characterization of human mitochondrial arginyl-tRNA synthetase*
**作者**：Wu J, et al.
**摘要概括**：
作者利用昆虫细胞系统重组表达了人源RARS2蛋白，解析其晶体结构，并揭示其催化结构域的关键氨基酸残基。通过体外酶动力学实验，阐明了RARS2催化精氨酰-tRNA合成的分子机制，为疾病相关突变的致病机理提供结构基础。
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**备注**：若需获取全文或更多文献，建议通过PubMed(https://pubmed.ncbi.nlm.nih.gov)或Google Scholar检索关键词“RARS2 recombinant protein”或结合具体研究主题(如酶活性、疾病模型)进一步筛选。
**Background of RARS2 Recombinant Protein**
RARS2 (mitochondrial arginyl-tRNA synthetase 2) is a nuclear-encoded enzyme critical for mitochondrial protein synthesis. It catalyzes the attachment of arginine to its cognate tRNA^(Arg) in the mitochondrial matrix, a step essential for translating mitochondrial DNA-encoded genes involved in oxidative phosphorylation (OXPHOS). Mutations in the *RARS2* gene are linked to pontocerebellar hypoplasia type 6 (PCH6), a severe neurodegenerative disorder characterized by impaired brain development, seizures, and progressive microcephaly, underscoring the enzyme's role in neuronal survival and energy metabolism.
The recombinant RARS2 protein is produced via heterologous expression systems (e.g., *E. coli* or mammalian cell cultures*) to study its structure, function, and interactions. Its production typically involves cloning the *RARS2* cDNA into expression vectors, followed by purification using affinity tags (e.g., His-tag). Recombinant RARS2 enables *in vitro* analyses, such as enzymatic activity assays, substrate specificity studies, and screening for potential therapeutic compounds targeting tRNA synthetase deficiencies.
Research on RARS2 recombinant protein also addresses mitochondrial disorders' molecular mechanisms, providing insights into how tRNA synthetase dysfunction disrupts OXPHOS and cellular energy balance. Additionally, it serves as a tool for developing gene therapy strategies or enzyme replacement therapies for PCH6 and related mitochondrial diseases. Despite progress, challenges remain in understanding its post-translational modifications, tissue-specific regulation, and interaction partners in mitochondrial translation. Further studies using recombinant RARS2 could unveil novel therapeutic avenues for mitochondrial pathologies.
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