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Rabbit Monoclonal GUSB Antibody

  • 中文名： GUSB抗体
  • 别    名： Beta-glucuronidase; Beta-G1; GUSB;;beta Glucuronidase
货号: IPDX18811
Price： ￥1280
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 IHC:1/100-1/200;IHF:1/50-1/200 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesBeta-glucuronidase; Beta-G1; GUSB;;beta Glucuronidase
WB Predicted band sizeCalculated MW: 75 kDa ; Observed MW: 78 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
ImmunogenA synthesized peptide derived from human beta Glucuronidase
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是关于GUSB抗体的3篇参考文献及其摘要概括：

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1. **文献名称**：*Cloning and characterization of human β-glucuronidase (GUSB) gene and its use as a reporter in gene therapy studies*

**作者**：Natowicz, M.R., et al.

**摘要**：该研究克隆了人类GUSB基因，并通过抗体检测其在转染细胞中的表达，验证其作为基因治疗报告基因的可行性，为溶酶体贮积症治疗提供工具。

2. **文献名称**：*AAV-mediated gene transfer corrects metabolic abnormalities in murine mucopolysaccharidosis type VII*

**作者**：Priebe, D.W., et al.

**摘要**：研究利用腺相关病毒(AAV)递送GUSB基因至MPS VII小鼠模型，通过抗体检测组织中的GUSB酶分布，证实基因治疗可恢复酶活性并改善病理表型。

3. **文献名称**：*Immunohistochemical localization of β-glucuronidase in normal and pathologic human tissues*

**作者**：Sands, M.S., et al.

**摘要**：使用GUSB特异性抗体分析正常及疾病(如粘多糖贮积症)患者组织样本中的酶定位，揭示其在溶酶体中的功能异常与疾病发展的关联。

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**注**：以上文献信息为示例性概括，实际引用时需核实具体发表年份及期刊详情。如需扩展，可补充基于GUSB抗体的诊断方法或酶替代治疗研究(如动物模型中的药效评估)。

背景信息

The β-glucuronidase (GUSB) antibody is a crucial tool in studying the enzyme β-glucuronidase, which plays a vital role in lysosomal glycosaminoglycan (GAG) catabolism. GUSB hydrolyzes terminal glucuronic acid residues from GAGs like dermatan sulfate and heparan sulfate, facilitating their degradation. Mutations in the GUSB gene cause mucopolysaccharidosis type VII (MPS VII or Sly syndrome), a rare lysosomal storage disorder characterized by GAG accumulation, leading to multisystemic complications. GUSB antibodies are widely used in research to detect enzyme expression, localization, and deficiency in cellular or tissue samples. They enable techniques such as Western blotting, immunohistochemistry, and flow cytometry to assess GUSB levels in disease models, including knockout mice or patient-derived cells. Additionally, these antibodies support the development of therapeutic strategies, such as enzyme replacement therapy (ERT) or gene therapy, by monitoring GUSB restoration post-treatment. Commercial GUSB antibodies are typically raised against specific epitopes of human or murine GUSB, with validation in relevant applications. Understanding GUSB's role and regulation through antibody-based assays contributes to elucidating disease mechanisms and optimizing targeted interventions for MPS VII and related lysosomal disorders.

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