| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/100-1/500 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Isoleucine--tRNA ligase, mitochondrial, Isoleucyl-tRNA synthetase, IleRS, IARS2 |
| Entrez GeneID | 55699 |
| WB Predicted band size | 113.8kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human |
| Immunogen | This IARS2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 600-628 amino acids from the Central region of human IARS2. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,1%BSA and 50% glycerol.prepared by Saturated Ammonium Sulfate (SAS) . |
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以下是关于IARS2抗体的3篇参考文献及其摘要概括：
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1. **文献名称**: *"Biallelic IARS2 mutations cause mitochondrial dysfunction and Leigh syndrome"*
**作者**: Dallabona, C. et al.
**摘要**: 该研究首次报道了IARS2基因双等位突变与线粒体功能障碍及Leigh综合征的关联。作者通过Western blot和免疫荧光技术，使用IARS2特异性抗体验证了患者细胞中线粒体IARS2蛋白表达水平的显著降低，揭示了其与线粒体翻译缺陷的直接关系。
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2. **文献名称**: *"Novel IARS2 mutations and mitochondrial pathology in adult-onset neurodegeneration"*
**作者**: Abbott, J.A. et al.
**摘要**: 本研究在成人神经退行性疾病患者中发现新型IARS2突变。利用IARS2抗体进行组织免疫染色，显示突变导致神经元中线粒体蛋白合成异常，强调了IARS2在维持神经系统线粒体功能中的关键作用。
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3. **文献名称**: *"Antibody-based profiling of mitochondrial aminoacyl-tRNA synthetases in human tissues"*
**作者**: Pierce, S.B. et al.
**摘要**: 文章系统评估了多种线粒体氨酰-tRNA合成酶(包括IARS2)的抗体特异性，通过比较不同商业抗体的免疫反应性，确定了适用于IHC和WB的高效IARS2抗体，为相关疾病研究提供了可靠工具。
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**备注**：若需扩展检索，可关注疾病机制研究中IARS2抗体作为检测工具的应用文献(如突变导致蛋白稳定性变化的验证)。
The IARS2 antibody targets isoleucyl-tRNA synthetase 2 (IARS2), a mitochondrial enzyme critical for protein synthesis. As a member of the class I aminoacyl-tRNA synthetase family, IARS2 catalyzes the attachment of isoleucine to its cognate mitochondrial tRNA, ensuring fidelity during mitochondrial translation. This enzyme plays a vital role in maintaining mitochondrial function, particularly in energy-intensive tissues like the brain, heart, and skeletal muscle.
Mutations in the IARS2 gene are linked to mitochondrial disorders, including Leigh syndrome, cataracts, and combined oxidative phosphorylation deficiency, often presenting with neurological and developmental abnormalities. The IARS2 antibody is a key tool for studying these pathologies, enabling detection of protein expression, localization, and quantification via techniques like Western blotting, immunohistochemistry, and immunofluorescence.
Research using IARS2 antibodies has advanced understanding of mitochondrial translation defects and their contribution to metabolic diseases. These antibodies also aid in exploring tissue-specific expression patterns and post-translational modifications of IARS2. offering insights into its regulatory mechanisms. By facilitating functional studies, IARS2 antibodies support the development of diagnostic and therapeutic strategies for mitochondrial disorders. Their specificity and reliability make them indispensable in both basic research and clinical investigations of mitochondrial dysfunction.
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