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Rabbit Monoclonal GLB1 Antibody

  • 中文名： GLB1抗体
  • 别    名： GLB1; ELNR1; Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin receptor 1
货号: IPDX21444
Price： ￥1280
数量：
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验证与应用

应用及物种
WB 1/500-1/1000 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 咨询技术 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesGLB1; ELNR1; Beta-galactosidase; Acid beta-galactosidase; Lactase; Elastin receptor 1
Entrez GeneID2720
WB Predicted band sizeCalculated MW: 76 kDa; Observed MW: 80 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 synthetic peptide of human GLB1
FormulationPurified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol.

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参考文献

以下是3条关于GLB1抗体的模拟参考文献(示例仅供参考，非真实文献)：

1. **文献名称**：*Development of a Monoclonal Antibody for Detection of GLB1 in GM1 Gangliosidosis*

**作者**：Brunetti-Pierri N, et al.

**摘要**：本研究报道了一种高特异性的抗GLB1单克隆抗体的开发，用于通过免疫印迹和免疫荧光技术检测患者成纤维细胞中GLB1蛋白的表达缺失，为GM1神经节苷脂贮积症的快速诊断提供了可靠工具。

2. **文献名称**：*GLB1 Enzyme Activity Restoration in a Mouse Model Using AAV-mediated Gene Therapy*

**作者**：Sena-Esteves M, et al.

**摘要**：通过腺相关病毒(AAV)载体递送功能性GLB1基因至疾病模型小鼠，利用抗GLB1抗体证实了靶组织中酶蛋白的恢复，显著改善了溶酶体贮积表型。

3. **文献名称**：*Structural Analysis of GLB1 Mutations by Epitope-specific Antibodies*

**作者**：Takano T, Itoh K.

**摘要**：通过设计针对GLB1不同结构域的多克隆抗体，揭示了致病突变对酶活性中心及稳定性的影响，为突变分类及个性化治疗策略提供了依据。

4. **文献名称**：*A Novel GLB1 Antibody-based ELISA for Newborn Screening of Lysosomal Storage Disorders*

**作者**：Annunziata I, et al.

**摘要**：开发了一种基于GLB1抗体的ELISA检测法，可定量分析干血斑中GLB1蛋白水平，初步验证显示其对新生儿GM1神经节苷脂贮积症筛查具有高灵敏度和特异性。

(注：以上内容为模拟生成，实际文献需通过学术数据库查询。)

背景信息

The GLB1 antibody targets β-galactosidase-1. a lysosomal enzyme encoded by the *GLB1* gene. This enzyme hydrolyzes terminal β-linked galactose residues in glycoproteins, glycolipids, and glycosaminoglycans, playing a critical role in lysosomal catabolism. Mutations in *GLB1* are linked to two autosomal recessive disorders: GM1 gangliosidosis (neurodegenerative) and Morquio B syndrome (skeletal dysplasia), both characterized by substrate accumulation due to enzymatic deficiency. GLB1 also exists in an alternative spliced form, elastin-binding protein (EBP), involved in elastogenesis and associated with vascular and connective tissue abnormalities. Antibodies against GLB1 are widely used in research to study lysosomal storage disorders, protein trafficking, and enzyme replacement therapies. They enable detection of GLB1 expression via Western blot, immunohistochemistry, or immunofluorescence, aiding in diagnostics and mechanistic studies. Additionally, GLB1 is studied in aging research, as senescence-associated β-galactosidase (SA-β-gal) activity, though debated, is often linked to lysosomal β-galactosidase isoforms. Commercially available GLB1 antibodies vary in specificity; some distinguish between the lysosomal enzyme and EBP, while others cross-react. Validation in knock-out models or siRNA-treated cells is essential to confirm target specificity. Overall, GLB1 antibodies are vital tools for exploring lysosomal biology, genetic diseases, and potential therapeutic interventions. (298 words)

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