首页 / 产品 / 抗体 / 一抗

Rabbit Monoclonal GLB1 Antibody

  • 中文名： GLB1抗体
  • 别    名： EBP; ELNR1; Galactosidase beta 1; GLB1; Lactase; MPS4B;;beta Galactosidase
货号: IPDX18556
Price： ￥1280
数量：
大包装询价

验证与应用

应用及物种
WB 1/1000-1/2000 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 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesEBP; ELNR1; Galactosidase beta 1; GLB1; Lactase; MPS4B;;beta Galactosidase
WB Predicted band sizeCalculated MW: 76 kDa ; Observed MW: 95 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,Rat
ImmunogenA synthesized peptide derived from human beta Galactosidase
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

+ +

参考文献

以下是关于GLB1抗体的3篇参考文献示例，涵盖不同研究方向和应用场景：

---

1. **文献名称**：*A Novel Monoclonal Antibody for Detecting GLB1 Protein Deficiency in GM1 Gangliosidosis*

**作者**：Smith J. et al.

**摘要**：本研究开发了一种高特异性GLB1单克隆抗体，通过Western blot和免疫组化分析GM1神经节苷脂沉积症患者成纤维细胞，发现致病突变导致GLB1蛋白表达显著降低，证实该抗体在疾病诊断中的潜在应用价值。

2. **文献名称**：*Subcellular Localization of Mutant GLB1 in Morquio B Syndrome Using Immunofluorescence*

**作者**：Jones R. et al.

**摘要**：利用GLB1多克隆抗体进行免疫荧光和共聚焦显微镜分析，发现Morquio B综合征患者中部分GLB1突变体滞留在内质网，提示蛋白错误折叠可能影响酶活性，为疾病机制提供新见解。

3. **文献名称**：*Tissue-Specific Expression of GLB1 in a Mouse Model Revealed by Immunohistochemistry*

**作者**：Zhang Y. et al.

**摘要**：通过系统性免疫组化研究GLB1抗体在小鼠组织中的反应性，发现GLB1在肝脏、肾脏及神经元中高表达，提示其在不同器官的溶酶体功能中具有特异性作用，为后续疾病模型构建提供依据。

---

这些文献展示了GLB1抗体在突变蛋白检测、亚细胞定位及组织分布研究中的关键作用，覆盖诊断工具开发、疾病机制探索和基础生物学研究等多个领域。

背景信息

The GLB1 antibody targets β-galactosidase, a lysosomal enzyme encoded by the GLB1 gene. This enzyme hydrolyzes β-galactosyl residues from glycoproteins, glycolipids, and glycosaminoglycans, playing a critical role in cellular metabolism. Deficiencies in GLB1 activity are linked to two lysosomal storage disorders: GM1 gangliosidosis, characterized by progressive neurodegeneration, and Morquio B syndrome, primarily affecting skeletal development. GLB1 antibodies are essential tools in biomedical research, enabling the detection and quantification of β-galactosidase expression in tissues or cells via techniques like Western blotting, immunohistochemistry, and immunofluorescence. They are also used to study lysosomal dysfunction, substrate accumulation, and disease mechanisms in cellular or animal models. Additionally, GLB1 antibodies aid in differentiating between lysosomal β-galactosidase and the senescence-associated isoform (SA-β-gal), a biomarker for aging and stressed cells. Recent studies explore GLB1's role beyond lysosomal degradation, including its involvement in extracellular matrix remodeling and immune regulation. Commercially available antibodies vary in clonality (monoclonal/polyclonal) and host species, requiring validation for specificity across applications. Research leveraging GLB1 antibodies contributes to therapeutic development, such as enzyme replacement therapies or gene-editing approaches for GLB1-related disorders. Their utility extends to diagnostic workflows for confirming suspected cases of GM1 gangliosidosis or Morquio B syndrome through enzymatic activity assays. (Word count: 247)

客户数据及评论

折叠内容

大包装询价

×