| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | SCEH |
| WB Predicted band size | 31 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, Rat |
| Immunogen | Fusion protein of human ECHS1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于ECHS1抗体的3篇参考文献及其摘要信息：
1. **文献名称**：*"ECHS1 mutations cause combined respiratory chain deficiency resulting in Leigh syndrome"*
**作者**：Haack, T.B. et al.
**摘要**：该研究通过全外显子测序发现ECHS1基因突变与线粒体能量代谢障碍相关，导致Leigh综合征(亚急性坏死性脑病)。作者使用ECHS1抗体进行蛋白质免疫印迹(Western blot)和免疫荧光染色，证实突变患者细胞中ECHS1蛋白表达显著降低，揭示了其在线粒体脂肪酸氧化及能量代谢中的关键作用。
2. **文献名称**：*"ECHS1 suppresses hepatocellular carcinoma by inhibiting inflammation via NF-κB/MAPK signaling pathways"*
**作者**：Wang, Y. et al.
**摘要**：本研究探讨ECHS1在肝癌中的抑癌机制。通过免疫组织化学(IHC)和Western blot分析肝癌组织样本，发现ECHS1表达下调与患者不良预后相关。实验表明，ECHS1抗体检测显示其通过抑制NF-κB/MAPK通路减少炎症反应，从而抑制肿瘤进展，为肝癌治疗提供潜在靶点。
3. **文献名称**：*"Validation of a novel ECHS1 antibody for functional studies in mitochondrial disorders"*
**作者**：Johnson, M.T. et al.
**摘要**：该文献报道了一种新型ECHS1抗体的开发和验证。作者通过质谱分析和敲除细胞模型证实抗体的特异性，并应用于免疫沉淀(IP)和免疫细胞化学实验。研究发现该抗体能高效检测ECHS1蛋白的亚细胞定位及表达水平，为代谢疾病研究提供了可靠工具。
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**说明**：
1. 以上文献聚焦于ECHS1抗体在疾病机制研究(如代谢障碍、癌症)及抗体开发中的应用，涵盖蛋白质表达分析、信号通路研究和抗体验证。
2. 研究多结合Western blot、免疫组化等技术，强调ECHS1在代谢和疾病中的功能及临床意义。
**Background of ECHS1 Antibody**
ECHS1 (Enoyl-CoA Hydratase Short Chain 1) is a mitochondrial enzyme involved in fatty acid β-oxidation, catalyzing the hydration of 2-trans-enoyl-CoA to 3-hydroxyacyl-CoA. It plays a critical role in energy metabolism and is essential for breaking down short-chain fatty acids and branched-chain amino acids. Dysregulation of ECHS1 has been linked to metabolic disorders, neurodegenerative diseases, and cancer, highlighting its physiological importance.
The ECHS1 antibody is a targeted immunological tool designed to detect and quantify ECHS1 protein expression in various biological samples. It is commonly produced in hosts like rabbits or mice using immunogenic peptides derived from conserved regions of the human ECHS1 protein. Validated for applications such as Western blotting, immunohistochemistry (IHC), immunofluorescence (IF), and flow cytometry, this antibody aids in studying ECHS1’s expression patterns, subcellular localization, and interactions in health and disease.
Research utilizing ECHS1 antibodies has provided insights into its role in mitochondrial dysfunction, metabolic reprogramming in cancer, and rare inborn errors of metabolism caused by ECHS1 mutations. These studies underscore its potential as a biomarker or therapeutic target. Quality validation, including knockout controls and cross-reactivity checks, ensures specificity, making the ECHS1 antibody a reliable reagent in both basic research and clinical investigations.
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