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Recombinant Human YARS2 protein

  • 中文名: 线粒体酪氨酸-tRNA合成酶(YARS2)重组蛋白
  • 别    名: YARS2;Tyrosine--tRNA ligase, mitochondrial
货号: PA1000-3505
Price: ¥询价
数量:
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产品详情

纯度>85%SDS-PAGE.
种属Human
靶点YARS2
Uniprot NoQ9Y2Z4
内毒素< 0.01EU/μg
表达宿主E.coli
表达区间17-477aa
氨基酸序列MGSSHHHHHH SSGLVPRGSH MTLNLSVLLP LGLRKAHSGA QGLLAAQKAR GLFKDFFPET GTKIELPELF DRGTASFPQT IYCGFDPTAD SLHVGHLLAL LGLFHLQRAG HNVIALVGGA TARLGDPSGR TKEREALETE RVRANARALR LGLEALAANH QQLFTDGRSW GSFTVLDNSA WYQKQHLVDF LAAVGGHFRM GTLLSRQSVQ LRLKSPEGMS LAEFFYQVLQ AYDFYYLFQR YGCRVQLGGS DQLGNIMSGY EFINKLTGED VFGITVPLIT STTGAKLGKS AGNAVWLNRD KTSPFELYQF FVRQPDDSVE RYLKLFTFLP LPEIDHIMQL HVKEPERRGP QKRLAAEVTK LVHGREGLDS AKRCTQALYH SSIDALEVMS DQELKELFKE APFSEFFLDP GTSVLDTCRK ANAIPDGPRG YRMITEGGVS INHQQVTNPE SVLIVGQHIL KNGLSLLKIG KRNFYIIKWL QL
预测分子量54 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.

参考文献

以下是关于YARS2重组蛋白的3篇代表性文献概览:

1. **"YARS2 mutations cause myopathy associated with lactic acidosis"**

*作者:Riley LG et al. (2013)*

摘要:报道YARS2基因突变与线粒体肌病及乳酸性酸中毒的关联,研究通过重组YARS2蛋白体外实验验证突变对酶活性的影响,揭示其在线粒体翻译中的关键作用。

2. **"Structural basis of pathogenicity of mitochondrial YARS2 mutations"**

*作者:Kohda M et al. (2016)*

摘要:解析重组人源YARS2蛋白的晶体结构,分析致病性突变(如Phe52Leu)对酶活性中心构象的影响,为理解相关线粒体疾病机制提供结构依据。

3. **"A yeast model for functional analysis of human YARS2 variants"**

*作者:Boniecki MT et al. (2017)*

摘要:建立酿酒酵母异源表达系统,利用重组YARS2蛋白验证人类疾病相关突变的互补功能,证实多个错义突变导致线粒体tRNA酪氨酸酰化缺陷。

注:实际文献检索建议通过PubMed/Google Scholar用关键词"YARS2 recombinant"、"YARS2 protein expression"查询最新研究,部分研究可能涉及重组蛋白在疾病建模或药物筛选中的应用扩展。

背景信息

**Background of YARS2 Recombinant Protein**

YARS2 (tyrosyl-tRNA synthetase 2. mitochondrial) is a nuclear-encoded enzyme critical for mitochondrial protein synthesis. As a member of the aminoacyl-tRNA synthetase (ARS) family, it catalyzes the attachment of tyrosine to its cognate mitochondrial tRNA (mt-tRNA^Tyr), ensuring the accuracy of translation within mitochondria. Unlike its cytosolic counterpart (YARS1), YARS2 is specifically imported into mitochondria, where it supports the production of oxidative phosphorylation (OXPHOS) complex subunits essential for cellular energy metabolism.

Mutations in the *YARS2* gene are linked to mitochondrial disorders, notably MLASA (mitochondrial myopathy, lactic acidosis, and sideroblastic anemia), characterized by muscle weakness, metabolic acidosis, and blood cell abnormalities. These pathogenic variants disrupt mitochondrial translation, impairing OXPHOS function and leading to tissue-specific energy deficits, particularly in high-demand organs like muscles and bone marrow.

Recombinant YARS2 protein is produced using biotechnological platforms (e.g., *E. coli* or mammalian expression systems) to study its structure, enzymatic activity, and interaction partners. This engineered protein serves as a tool to investigate disease mechanisms, screen potential therapeutics, or validate genetic variants. Recent studies also explore its role beyond translation, including mitochondrial stress signaling and retrograde communication with the nucleus.

Research on YARS2 recombinant protein advances understanding of mitochondrial biology and offers pathways for targeted therapies, such as enzyme replacement or gene editing, to address YARS2-related disorders. Its study further contributes to the broader field of ARS-associated diseases, highlighting the intersection of mitochondrial dysfunction and human pathology.

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