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Anti-GLA/alpha-Galactosidase A Antibody (2S483)
还可以产品编号 TMAY-02227
Anti-GLA/alpha-Galactosidase A Antibody (2S483) 是一种 Rabbit 抗体，靶向 GLA/alpha-Galactosidase A。Anti-GLA/alpha-Galactosidase A Antibody (2S483) 可用于 WB。
Anti-GLA/alpha-Galactosidase A Antibody (2S483)
Anti-GLA/alpha-Galactosidase A Antibody (2S483)
还可以产品编号 TMAY-02227
Anti-GLA/alpha-Galactosidase A Antibody (2S483) 是一种 Rabbit 抗体，靶向 GLA/alpha-Galactosidase A。Anti-GLA/alpha-Galactosidase A Antibody (2S483) 可用于 WB。
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抗原信息
| 产品描述 | Anti-GLA/alpha-Galactosidase A Antibody (2S483) is a Rabbit antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (2S483) can be used in WB. |
| Ig Type | Monoclonal Rabbit IgG |
| 克隆号 | 2S483 |
| 交叉反应 | Human |
| 验证活性 | Anti-GLA rabbit monoclonal antibody at 1:500 dilution. -Lane A: MCF7 Whole Cell Lysate. -Lane B: 293T Whole Cell lysate. -Lysates/proteins at 30 μg per lane. -Secondary -Goat Anti-Rabbit IgG H&L (Dylight800) at 1/10000 dilution. -Developed using the Odyssey technique. -Performed under reducing conditions. -Predicted band size:49 kDa. -Observed band size:49 kDa |
| 应用 | WB |
| 推荐剂量 | WB: 1:500-1:2000 |
| 抗体种类 | Monoclonal |
| 宿主来源 | Rabbit |
| 构建方式 | This antibody was obtained from a rabbit immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; TMPY-01727; NP_000160.1; Met1-Leu429). |
| 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 / GLA 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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