| 货号 | AF6146-SP |
| 别名 | agalsidase alfa; Agalsidase alpha; Agalsidase; Alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1; Alpha-D-galactoside galactohydrolase; alpha-gal A; alpha-galactosidase A; EC 3.2.1; EC 3.2.1.22; GALA; galactosidase, alpha; Melibiase |
| 反应种属 | Human |
| 应用 | Immunocytochemistry(5-15 µg/mL) |
| 目标/特异性 | Detects human alpha ‑Galactosidase A/GLA in direct ELISAs. |
| 使用方法 | Immunocytochemistry: 5-15 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 | 2717 (Human) |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | Chinese hamster ovary cell line CHO-derived recombinant human alpha ‑Galactosidase A/GLA Leu32-Leu429 Accession # P06280 |
| 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 | Human alpha -GalactosidaseA is a homodimeric glycoprotein that can release terminal alpha -galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose (1). It is a lysosomal enzyme and is responsible for degradation of glycolipid globotriaosylceramide (Gb3) (Gal alpha 1‑4Gal beta 1‑4Glc beta ‑ceramide). Mutations in this gene cause Fabry disease, an X-linked hereditary lysosomal storagedisease with the accumulation of Gb3 in the walls of small blood vessels,nerves, dorsal root ganglia, renal glomerular and tubular epithelialcells, and cardiomyocytes (2, 3). Inability to prevent the glycosphingolipid deposition can cause hypertension, strokes, heart attack and progressive renal failure (4). Current treatment for Fabry disease is enzyme replacement therapy using intravenously delivered recombinant alpha -Galactosidase A (5, 6). |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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alpha ‑Galactosidase A/GLA in HeLa Human Cell Line. alpha ‑Galactosidase A/GLA was detected in immersion fixed HeLa human cervical epithelial carcinoma cell line using Sheep Anti-Human alpha ‑Galactosidase A/GLA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6146) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # NL010). LAMP1 was also detected using Mouse Anti-Human LAMP1 Monoclonal Antibody (Catalog # MAB4800). Cells were stained using the NorthernLights™ 493-conjugated Anti-Mouse IgG Secondary Antibody (green; Catalog # NL009). Cells were counterstained with DAPI (blue). Specific staining was localized to lysosomes. View our protocol for Fluorescent ICC Staining of Cells on Coverslips. |
