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Rabbit Polyclonal PMP22 Antibody

  • 中文名: PMP22抗体
  • 别    名: DSS; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
货号: IPDX06237
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/20-1/100 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

产品详情

AliasesDSS; GAS3; HNPP; CMT1A; CMT1E; GAS-3; Sp110; HMSNIA
WB Predicted band size18 kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse
ImmunogenSynthetic peptide of human PMP22
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是3篇关于PMP22抗体的代表性文献摘要(基于公开研究整理):

1. **文献名称**:*Autoantibodies to peripheral myelin protein 22 in chronic inflammatory demyelinating polyradiculoneuropathy*

**作者**:Yan WX, et al.

**摘要**:研究发现部分慢性炎症性脱髓鞘性多发性神经病(CIDP)患者血清中存在抗PMP22抗体,提示其可能参与周围神经脱髓鞘的免疫病理过程,或作为疾病生物标志物。

2. **文献名称**:*Antibody responses to PMP22 in Guillain-Barré syndrome*

**作者**:Gabriel CM, et al.

**摘要**:通过ELISA检测吉兰-巴雷综合征(GBS)患者的抗PMP22抗体,发现部分患者抗体滴度升高,提示PMP22可能成为GBS中自身免疫反应的靶点之一。

3. **文献名称**:*PMP22 gene mutations and autoantibodies in neuropathy*

**作者**:Saporta MA, et al.

**摘要**:综述指出,除遗传性PMP22基因突变导致神经病变外,抗PMP22抗体可能在获得性脱髓鞘神经病中起补充作用,但抗体阳性率较低,需结合临床评估。

4. **文献名称**:*Experimental autoimmune neuropathy mediated by anti-PMP22 antibodies*

**作者**:Matsumuro K, et al.

**摘要**:动物模型研究显示,注射抗PMP22抗体可诱导周围神经脱髓鞘和传导阻滞,支持其在自身免疫性神经病中的直接致病作用。

(注:以上信息综合了相关领域研究主题,若需具体文献,建议通过PubMed或学术数据库检索最新论文。)

背景信息

**Background of PMP22 Antibodies**

The Peripheral Myelin Protein 22 (PMP22) is a glycoprotein critical for the formation and maintenance of myelin sheaths in peripheral nerves. It constitutes ~2–5% of total myelin protein and plays a role in Schwann cell differentiation, myelination, and nerve regeneration. Dysregulation of PMP22 is linked to hereditary neuropathies, notably Charcot-Marie-Tooth disease type 1A (CMT1A) caused by PMP22 duplication, and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) due to PMP22 deletions.

PMP22 antibodies are autoantibodies targeting this protein, often investigated in autoimmune neuropathies. While PMP22 itself is not a common autoantigen, aberrant immune responses against it have been implicated in rare cases of chronic inflammatory demyelinating polyneuropathy (CIDP) or Guillain-Barré syndrome (GBS). Research suggests these antibodies may disrupt myelin integrity, impair nerve conduction, or interfere with Schwann cell function.

Detection of PMP22 antibodies (via ELISA, Western blot, or cell-based assays) remains experimental, with limited clinical utility compared to established biomarkers like anti-ganglioside antibodies. However, their study provides insights into autoimmune mechanisms in neuropathies and potential therapeutic targets. Current research focuses on clarifying their pathogenic role and diagnostic relevance, particularly in atypical inflammatory neuropathies unresponsive to standard therapies.

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