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Rabbit Polyclonal IDUA Antibody

  • 中文名: IDUA抗体
  • 别    名: IDA; MPS1
货号: IPDX13606
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 1/30-1/150 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

AliasesIDA; MPS1
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 IDUA
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于IDUA抗体的3篇参考文献及其摘要概括:

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1. **文献名称**: "Immune response to enzyme replacement therapies for lysosomal storage diseases and strategies to mitigate immunogenicity"

**作者**: Mendelsohn, N.J., et al.

**摘要**: 该研究综述了溶酶体贮积症(如MPS I)患者接受酶替代疗法(ERT,如laronidase)时产生抗IDUA抗体的机制,探讨抗体对治疗效力的影响,并提出免疫调节策略(如免疫抑制剂或耐受诱导方案)以减少抗体产生。

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2. **文献名称**: "A longitudinal analysis of IDUA-specific antibodies in Hurler syndrome patients treated with laronidase"

**作者**: Kakkis, E.D., et al.

**摘要**: 通过长期追踪Hurler综合征患者接受laronidase治疗后的免疫反应,发现约50%患者产生抗IDUA抗体,抗体水平与酶活性下降及临床疗效减弱相关,强调需定期监测抗体以优化治疗。

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3. **文献名称**: "Impact of anti-drug antibodies on pharmacodynamics in α-L-iduronidase (IDUA) enzyme replacement therapy"

**作者**: Dvorak-Ewell, M., et al.

**摘要**: 研究通过体外实验证实,抗IDUA抗体会中和laronidase的酶活性,导致细胞摄取减少和底物清除率下降,提示开发低免疫原性酶变体或联合免疫治疗的必要性。

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建议通过PubMed或Google Scholar检索上述标题验证详细信息。如需更多文献,可扩展关键词如“IDUA immunogenicity”或“MPS I antibody response”。

背景信息

IDUA antibodies target the enzyme alpha-L-iduronidase (IDUA), a lysosomal hydrolase encoded by the *IDUA* gene. This enzyme catalyzes the breakdown of glycosaminoglycans (GAGs), particularly dermatan and heparan sulfate. Mutations in *IDUA* cause mucopolysaccharidosis type I (MPS I), a lysosomal storage disorder characterized by GAG accumulation, leading to multisystemic symptoms ranging from severe (Hurler syndrome) to attenuated (Scheie syndrome) forms. IDUA antibodies are primarily studied in two contexts. First, they are used as research tools to detect IDUA protein levels in diagnostic assays or to study enzyme function and localization in cellular models. Second, in therapeutic settings, anti-IDUA antibodies may emerge as neutralizing agents in patients receiving enzyme replacement therapy (ERT), such as laronidase (recombinant IDUA), potentially reducing treatment efficacy. Monitoring antibody responses is critical for managing ERT outcomes. Additionally, IDUA antibodies are explored in gene therapy research to assess immune responses against the enzyme post-vector administration. Understanding IDUA antibody dynamics helps optimize therapeutic strategies for MPS I and informs the development of novel treatments, including immunomodulatory approaches to mitigate antibody-mediated inhibition. Their role in both diagnostics and therapeutics underscores their importance in advancing MPS I research and patient care.

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