| 纯度 | >90%SDS-PAGE. |
| 种属 | Human |
| 靶点 | EARS2 |
| Uniprot No | Q5JPH6 |
| 内毒素 | < 0.01EU/μg |
| 表达宿主 | E.coli |
| 表达区间 | 1-523aa |
| 氨基酸序列 | MAALLRRLLQRERPSAASGRPVGRREANLGTDAGVAVRVRFAPSPTGFLHLGGLRTALYNYIFAKKYQGSFILRLEDTDQTRVVPGAAENIEDMLEWAGIPPDESPRRGGPAGPYQQSQRLELYAQATEALLKTGAAYPCFCSPQRLELLKKEALRNHQTPRYDNRCRNMSQEQVAQKLAKDPKPAIRFRLEQVVPAFQDLVYGWNRHEVASVEGDPVIMKSDGFPTYHLACVVDDHHMGISHVLRGSEWLVSTAKHLLLYQALGWQPPHFAHLPLLLNRDGSKLSKRQGDVFLEHFAADGFLPDSLLDIITNCGSGFAENQMGRTLPELITQFNLTQVTCHSALLDLEKLPEFNRLHLQRLVSNESQRRQLVGKLQVLVEEAFGCQLQNRDVLNPVYVERILLLRQGHICRLQDLVSPVYSYLWTRPAVGRAQLDAISEKVDVIAKRVLGLLERSSMSLTQDMLNGELKKLSEGLEGTKYSNVMKLLRMALSGQQQGPPVAEMMLALGPKEVRERIQKVVSS |
| 预测分子量 | 58,6 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EARS2重组蛋白的示例文献摘要(注：内容为示例性概括，非真实文献)：
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1. **文献名称**：*Recombinant EARS2 Protein Expression and Functional Characterization in Mitochondrial Translation*
**作者**：Lee, J. et al.
**摘要**：本研究通过在大肠杆菌系统中高效表达并纯化重组人源EARS2蛋白，验证其作为线粒体谷氨酰-tRNA合成酶的活性，证实其在维持线粒体翻译及呼吸链复合体组装中的关键作用。
2. **文献名称**：*Structural Insights into EARS2 Mutations Associated with Leukodystrophy*
**作者**：Gomez, M. et al.
**摘要**：通过X射线晶体学解析重组EARS2蛋白的催化结构域，揭示了致病性突变(如p.Arg228Gly)如何破坏酶与tRNA的相互作用，导致婴儿期发病的脑白质营养不良综合征。
3. **文献名称**：*EARS2 Deficiency: Recombinant Protein-Based Diagnostic Assay Development*
**作者**：Chen, X. et al.
**摘要**：开发基于重组EARS2蛋白的体外酶活性检测方法，用于快速筛查临床疑似线粒体疾病患者，证明其在诊断EARS2基因突变相关神经退行性疾病中的灵敏性和特异性。
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**注**：以上为模拟内容，实际文献需通过PubMed/Google Scholar等平台以关键词“EARS2 recombinant”或“EARS2 glutamyl-tRNA synthetase”检索。
**Background of EARS2 Recombinant Protein**
EARS2 (Glutamyl-tRNA Synthetase 2. Mitochondrial) is a nuclear-encoded enzyme critical for mitochondrial protein synthesis. It belongs to the family of aminoacyl-tRNA synthetases (aaRSs), which catalyze the attachment of specific amino acids to their cognate tRNAs during translation. EARS2 specifically charges mitochondrial tRNA-glutamate (tRNAᴳˡᵘ) with glutamic acid, a step essential for the accurate decoding of mitochondrial mRNA and subsequent synthesis of oxidative phosphorylation (OXPHOS) complex subunits.
The EARS2 gene is located on chromosome 16 and encodes a protein that undergoes post-translational processing, including cleavage of its N-terminal mitochondrial targeting sequence. Mature EARS2 functions within the mitochondrial matrix, interacting with other aaRSs and translation factors. Mutations in EARS2 are linked to mitochondrial disorders, such as Combined Oxidative Phosphorylation Deficiency 12 (COXPD12), characterized by encephalopathy, hypotonia, and lactic acidosis. These pathologies arise from impaired mitochondrial translation, leading to defective OXPHOS complexes and compromised cellular energy production.
Recombinant EARS2 protein is engineered using heterologous expression systems (e.g., *E. coli* or mammalian cells*) to study its structure, enzymatic activity, and role in disease. Purified recombinant EARS2 enables biochemical assays, structural analysis (e.g., X-ray crystallography), and screening for therapeutic compounds targeting aaRS-related disorders. Its production also supports functional studies to dissect the molecular mechanisms of pathogenic mutations and explore rescue strategies, such as gene therapy or small-molecule chaperones.
Research on EARS2 recombinant protein contributes to understanding mitochondrial translation fidelity, neurodegenerative disease pathways, and potential interventions for mitochondrial dysfunction. Its applications span basic science, diagnostic tool development, and preclinical drug discovery.
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