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Mouse Monoclonal CHRND Antibody

  • 中文名: CHRND抗体
  • 别    名: ACHRD; CMS2A; CMS3A; CMS3B; CMS3C; FCCMS; SCCMS
货号: IPD31349
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 1/200 - 1/400 Human,Mouse,Rat
Elisa 1/10000 Human,Mouse,Rat

产品详情

AliasesACHRD; CMS2A; CMS3A; CMS3B; CMS3C; FCCMS; SCCMS
Entrez GeneID1144
clone1H1F9
WB Predicted band size58.8kDa
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
ImmunogenPurified recombinant fragment of human CHRND (AA: extra 22-245) expressed in E. Coli.
FormulationPurified antibody in PBS with 0.05% sodium azide

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参考文献

以下是关于CHRND抗体的假设性参考文献示例(仅供参考,建议通过学术数据库核实最新研究):

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1. **标题**: *Autoantibodies Targeting the Nicotinic Acetylcholine Receptor δ Subunit in Seronegative Myasthenia Gravis*

**作者**: Smith A, et al.

**摘要**: 研究在血清阴性重症肌无力(MG)患者中发现了针对AChR δ亚基(CHRND)的自身抗体。通过细胞实验证实,这些抗体可阻断神经肌肉信号传递,提示其可能参与MG的病理机制,尤其在常规抗体检测阴性的病例中。

2. **标题**: *Functional Impact of CHRND-Specific Antibodies in Congenital Myasthenic Syndromes*

**作者**: Jones R, Lee C.

**摘要**: 分析了一组先天性肌无力综合征(CMS)患者的CHRND基因突变与抗体产生的关系,发现部分患者存在抗δ亚基抗体,可能通过竞争性抑制乙酰胆碱结合位点加剧症状。

3. **标题**: *Experimental Induction of Neuromuscular Dysfunction via CHRND Antibody Transfer in Mice*

**作者**: Wilson K, et al.

**摘要**: 在小鼠模型中,注射抗CHRND抗体导致肌肉无力及突触后膜AChR密度下降,模拟人类自身免疫性MG表型,为抗体介导的病理机制提供了直接证据。

4. **标题**: *Clinical Heterogeneity in Myasthenia Gravis Patients with CHRND Antibodies*

**作者**: Brown T, et al.

**摘要**: 报道了6例携带抗CHRND抗体的MG患者,其临床表现包括眼肌无力和全身性症状,部分患者对利妥昔单抗治疗反应良好,提示抗体亚型可能影响治疗策略。

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**备注**:以上为假设性示例,实际文献需通过PubMed或Google Scholar检索关键词(如“CHRND antibody myasthenia”)获取。近年来关于CHRND抗体的研究较少,多数聚焦于基因突变,建议结合“acetylcholine receptor delta subunit”和“autoantibody”等术语扩展搜索。

背景信息

The CHRND gene encodes the delta subunit of the nicotinic acetylcholine receptor (nAChR), a ligand-gated ion channel critical for neuromuscular signal transmission. nAChRs are pentameric proteins composed of α, β, γ (or ε in adults), and δ subunits, forming a channel that depolarizes the postsynaptic membrane upon acetylcholine binding, triggering muscle contraction. The δ subunit (CHRND) is essential for receptor assembly and function. Antibodies targeting CHRND are valuable tools for studying neuromuscular junction disorders, such as congenital myasthenic syndromes (CMS) or autoimmune myasthenia gravis (MG), where disrupted receptor activity causes muscle weakness. In research, CHRND antibodies are used in techniques like Western blot, immunohistochemistry, or flow cytometry to localize or quantify receptor expression, assess developmental changes (e.g., γ-to-ε subunit switch), or investigate disease mechanisms. Autoantibodies against CHRND in MG block receptor function, impairing synaptic signaling. Additionally, CHRND mutations linked to CMS highlight its role in receptor stability and ion conductance. Studying CHRND antibodies aids in understanding receptor biology, diagnosing neuromuscular pathologies, and developing targeted therapies.

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