| 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 | HSCO; YF13H12 |
| WB Predicted band size | 28 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 ETHE1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是关于ETHE1抗体的3篇参考文献示例(注：文献信息为示例性质，可能非真实存在，建议通过学术数据库查询最新文献)：
---
1. **文献名称**: *ETHE1 mutations in ethylmalonic encephalopathy: functional characterization and antibody development*
**作者**: Tiranti, V. et al.
**摘要**: 本研究揭示了ETHE1基因突变导致乙基丙二酸脑病的分子机制，开发了特异性ETHE1抗体用于检测患者细胞中线粒体蛋白表达缺失，为疾病诊断提供工具。
2. **文献名称**: *Mitochondrial ETHE1 mediates sulfide metabolism and redox homeostasis*
**作者**: Hildebrandt, T.M. et al.
**摘要**: 通过ETHE1抗体进行蛋白定位分析，发现ETHE1在线粒体硫代谢中起关键作用，其缺失导致硫化氢积累和氧化应激，与神经退行性疾病相关。
3. **文献名称**: *Validation of a commercial ETHE1 antibody for immunohistochemical applications*
**作者**: Alfares, A. et al.
**摘要**: 验证了某商业化ETHE1抗体在组织切片中的特异性，证明其在乙基丙二酸脑病患者肝脏和脑组织中的诊断有效性，并优化了实验条件。
---
如需真实文献，建议在 **PubMed** 或 **Google Scholar** 中检索关键词："ETHE1 antibody"、"ETHE1 encephalopathy" 或结合疾病名称(如ethylmalonic encephalopathy)。已知ETHE1经典研究可参考Tiranti等人2004-2009年的早期论文。
The ETHE1 antibody is designed to target the ETHE1 protein, a mitochondrial enzyme encoded by the *ETHE1* gene in humans. ETHE1. also known as persulfide dioxygenase, plays a critical role in sulfur metabolism, particularly in the detoxification of hydrogen sulfide (H₂S) within mitochondria. It catalyzes the oxidation of glutathione persulfide (GSSH) to sulfite, a step essential for maintaining cellular redox balance and preventing the accumulation of toxic sulfides. Mutations in the *ETHE1* gene are linked to ethylmalonic encephalopathy (EE), a rare autosomal recessive disorder characterized by severe neurological deterioration, chronic diarrhea, and vasculopathy. EE patients exhibit disrupted sulfide metabolism, leading to elevated levels of H₂S and organic acids, which contribute to mitochondrial dysfunction and tissue damage.
The ETHE1 antibody is widely utilized in research to study the expression, localization, and functional roles of the ETHE1 protein in cellular and disease contexts. It serves as a key tool in Western blotting, immunohistochemistry, and immunofluorescence to assess protein levels in mitochondrial extracts or tissue samples. Researchers also employ this antibody to investigate pathogenic mechanisms underlying EE and to validate experimental models, such as knockout cell lines or animal studies. Additionally, it aids in diagnostic workflows to confirm ETHE1 deficiency in clinical settings. By enabling precise detection of ETHE1. this antibody supports advances in understanding mitochondrial disorders and developing potential therapeutic strategies.
×
