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Anti-GLA/alpha-Galactosidase A Antibody (5V259)
还可以产品编号 TMAY-02225
Anti-GLA/alpha-Galactosidase A Antibody (5V259) 是一种 Mouse 抗体，靶向 GLA/alpha-Galactosidase A。Anti-GLA/alpha-Galactosidase A Antibody (5V259) 可用于 ELISA,ELISA(Cap)。
Anti-GLA/alpha-Galactosidase A Antibody (5V259)
Anti-GLA/alpha-Galactosidase A Antibody (5V259)
还可以产品编号 TMAY-02225
Anti-GLA/alpha-Galactosidase A Antibody (5V259) 是一种 Mouse 抗体，靶向 GLA/alpha-Galactosidase A。Anti-GLA/alpha-Galactosidase A Antibody (5V259) 可用于 ELISA,ELISA(Cap)。
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产品介绍
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偶联与修饰
抗原信息
| 产品描述 | Anti-GLA/alpha-Galactosidase A Antibody (5V259) is a Mouse antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (5V259) can be used in ELISA,ELISA(Cap). |
| Ig Type | Monoclonal Mouse IgG1 |
| 克隆号 | 5V259 |
| 交叉反应 | Human |
| 应用 | ELISA,ELISA(Cap) |
| 抗体种类 | Monoclonal |
| 宿主来源 | Mouse |
| 构建方式 | This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human alpha-Galactosidase A (rh alpha-Galactosidase A; TMPY-01727; NP_000160.1; Met1-Leu429). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography. |
| 纯化方式 | Protein A |
| 性状 | Liquid |
| 缓冲液 | 0.2 μm filtered solution in PBS |
| 研究背景 | Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction. |
| 偶联 | Unconjugated |
| 免疫原 | Recombinant Human alpha-Galactosidase A Protein (TMPY-01727) |
| 抗原种属 | Human |
存储&运输
| 储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free. |
| 运输方式 | Shipping with blue ice. |
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比如您的给药剂量是 10 mg/kg ，每只动物体重 20 g ，给药体积 100 μL ， 
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