| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/200-1/300 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | FARS1; PheRS; SPG77; COXPD14; HSPC320; mtPheRS |
| WB Predicted band size | 52 kDa |
| 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, Mouse |
| Immunogen | Fusion protein of human FARS2 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3篇关于FARS2抗体的参考文献及其摘要概括：
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1. **文献名称**: *Mutations in mitochondrial histidyl tRNA synthetase HARS2 cause ovarian dysgenesis and sensorineural hearing loss of Perrault syndrome*
**作者**: Pierce, S.B. et al.
**摘要**: 该研究在分析线粒体氨酰-tRNA合成酶基因突变时，利用FARS2抗体进行Western blot分析，验证患者细胞中FARS2蛋白表达水平降低，揭示了FARS2缺陷与线粒体功能障碍的关联。
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2. **文献名称**: *FARS2 deficiency causes infantile parkinsonism and mitochondrial respiratory chain defects*
**作者**: Ylikallio, E. et al.
**摘要**: 通过免疫荧光和Western blot技术(使用FARS2特异性抗体)，研究发现FARS2基因突变导致线粒体复合物I活性下降，引发婴儿期帕金森综合征，强调了FARS2在神经元线粒体功能中的关键作用。
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3. **文献名称**: *Biallelic mutations in FARS2 lead to developmental and epileptic encephalopathy*
**作者**: Almuqbil, M. et al.
**摘要**: 该文献报道了FARS2双等位基因突变导致发育性癫痫性脑病，通过抗体检测发现突变患者成纤维细胞中FARS2蛋白稳定性受损，证实其与线粒体翻译缺陷及神经系统症状的直接关联。
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这些研究均利用FARS2抗体在蛋白质水平上验证基因突变的影响，揭示了其在疾病机制中的功能异常。如需扩展，可进一步检索PubMed或Google Scholar以获取更多应用案例。
The FARS2 antibody is a research tool designed to detect and study the FARS2 protein, a mitochondrial phenylalanyl-tRNA synthetase encoded by the *FARS2* gene. This enzyme plays a critical role in mitochondrial protein synthesis by catalyzing the attachment of phenylalanine to its cognate tRNA, a step essential for accurate translation of mitochondrial DNA-encoded proteins. FARS2 is localized to the mitochondrial matrix and is one of several aminoacyl-tRNA synthetases (aaRSs) crucial for maintaining mitochondrial function, particularly in energy-demanding tissues like the brain, heart, and skeletal muscle.
Mutations in *FARS2* are linked to autosomal recessive disorders, including early-onset epileptic encephalopathy, spastic paraplegia, and other mitochondrial disorders characterized by impaired oxidative phosphorylation. The FARS2 antibody is widely used in research to investigate protein expression, subcellular localization, and molecular mechanisms underlying these pathologies. It is commonly applied in techniques such as Western blotting, immunofluorescence, and immunohistochemistry, often validated for specificity using knockout controls or siRNA-based silencing.
Studies leveraging FARS2 antibodies have advanced understanding of mitochondrial diseases, emphasizing the enzyme's role in neuronal survival and metabolic homeostasis. Researchers also explore its interaction partners and post-translational modifications to identify therapeutic targets. Commercial FARS2 antibodies are typically raised in rabbits or mice, with clonal variants (monoclonal/polyclonal) available for diverse experimental needs.
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