| 货号 | AF6464-SP |
| 别名 | Acid beta-galactosidase; beta-galactosidase; EBP; EC 3.2.1.23; elastin receptor 1 (67kD); Elastin receptor 1; elastin receptor 1, 67kDa; ELNR1; galactosidase, beta 1; GLB1; Lactase; MPS4B |
| 反应种属 | Human |
| 应用 | Western Blot(1 µg/mL) Simple Western(10 µg/mL) |
| 目标/特异性 | Detects human beta ‑Galactosidase‑1/GLB1 in direct ELISAs and Western blots. |
| 使用方法 | Western Blot: 1 µg/mL Simple Western: 10 µg/mL |
| 来源 | Sterile PBS to a final concentration of 0.2 mg/mL. |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 2720 (Human) |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | Chinese hamster ovary cell line CHO-derived recombinant human beta ‑Galactosidase‑1/GLB1 Leu24-Val677 Accession # AAA51819 |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Sterile PBS to a final concentration of 0.2 mg/mL. |
| 背景 | GLB1, a 60-76 kDa (predicted) glycoprotein, is a lysosomal beta ‑galactosidase that hydrolyzes the terminal beta -galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome (also known as mucopolysaccharidosis IVB) (1, 2, 3). In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0±3% of normal) of lysosomal beta ‑galactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate (4). More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases (5). In lysosome, the mature beta -galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex (6). An alternative splicing at the RNA level of GLB1 results a catalytically inactive beta -galactosidase (also called elastin-binding protein) that plays an important role in vascular development (7). |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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Detection of Human beta ‑Galactosidase‑1/GLB1 by Western Blot. Western blot shows lysates of JEG‑3 human epithelial choriocarcinoma cell line and KG‑1 human acute myelogenous leukemia cell line. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human beta ‑Galactosidase‑1/GLB1 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6464) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). Specific bands were detected for beta ‑Galactosidase‑1/GLB1 at approximately 60 kDa and 80 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 8. | |
Detection of Human 12-230 kDa separation system. |
