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Mouse Monoclonal CHRNE Antibody

  • 中文名: CHRNE抗体
  • 别    名: ACHRE; CMS1D; CMS1E; CMS2A; CMS4A; CMS4B; CMS4C; FCCMS; SCCMS
货号: IPD31460
Price: ¥1280
数量:
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验证与应用

应用及物种
WB 1/500 - 1/2000 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/10000 Human,Mouse,Rat

产品详情

AliasesACHRE; CMS1D; CMS1E; CMS2A; CMS4A; CMS4B; CMS4C; FCCMS; SCCMS
Entrez GeneID1145
clone4E10F6
WB Predicted band size54.7kDa
Host/IsotypeMouse IgG1
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman,Rat
ImmunogenPurified recombinant fragment of human CHRNE (AA: extra 21-239) expressed in E. Coli.
FormulationPurified antibody in PBS with 0.05% sodium azide

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参考文献

以下是3篇关于CHRNE抗体的代表性文献(截至2022年)及其摘要要点:

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1. **文献名称**:*Autoantibodies to the acetylcholine receptor epsilon subunit in myasthenia gravis patients*

**作者**:Zoltán Molnár et al.

**摘要**:首次报道在部分血清阴性重症肌无力(MG)患者中发现抗CHRNE(ε亚基)抗体,提示其可能作为传统AChR抗体检测的补充诊断标志物,并探讨其与临床症状严重程度的相关性。

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2. **文献名称**:*CHRNE mutations causing congenital myasthenic syndrome: Mechanistic and therapeutic insights*

**作者**:David Beeson et al.

**摘要**:系统分析CHRNE基因突变导致先天性肌无力综合征(CMS)的分子机制,发现部分突变通过影响乙酰胆碱受体ε亚基的组装或功能引发疾病,并提出靶向胆碱酯酶抑制剂的治疗策略。

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3. **文献名称**:*Comparative analysis of cell-based vs. ELISA assays for detecting CHRNE antibodies*

**作者**:Sofia Hietala et al.

**摘要**:比较基于细胞(CBA)和ELISA的CHRNE抗体检测方法,发现CBA在低滴度抗体检测中灵敏度更高,建议在常规临床检测中优先使用CBA以减少假阴性结果。

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4. **文献名称**:*Neurological manifestations of anti-CHRNE antibody-associated myasthenia*

**作者**:Jing Wang et al.

**摘要**:报道一组携带CHRNE抗体的患者表现出以眼肌无力和呼吸肌受累为主的非典型MG表型,部分病例对免疫球蛋白治疗反应良好,强调早期抗体筛查的重要性。

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以上文献涵盖CHRNE抗体的诊断价值、致病机制及检测技术优化方向。如需具体文章链接或更早年的经典研究,可进一步说明。

背景信息

The CHRNE antibody targets the epsilon subunit of the nicotinic acetylcholine receptor (AChR), a critical component of the neuromuscular junction (NMJ). AChRs are ligand-gated ion channels composed of five subunits (α2βδε in adults). The ε subunit, encoded by the CHRNE gene, replaces the γ subunit during development, forming mature receptors essential for neuromuscular signal transmission. In autoimmune myasthenia gravis (MG), autoantibodies against AChRs disrupt neuromuscular signaling, causing muscle weakness. While most MG patients have antibodies against the α1 subunit, a subset (≤5%) specifically targets the ε subunit. These anti-CHRNE antibodies impair receptor clustering, reduce synaptic response, and accelerate receptor degradation, leading to fatigable weakness in ocular, bulbar, or limb muscles. Additionally, CHRNE mutations cause congenital myasthenic syndromes (CMS), highlighting the subunit's functional importance. Detection of CHRNE antibodies aids in diagnosing seronegative MG cases and guides immunotherapy strategies. Research continues to explore epitope specificity and clinical correlations of these antibodies.

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