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Anti-Galactosidase alpha Polyclonal Antibody 是一种 Rabbit 抗体,靶向 Galactosidase alpha。Anti-Galactosidase alpha Polyclonal Antibody 可用于 IHC-P,IHC-Fr,IF。
Anti-Galactosidase alpha Polyclonal Antibody 是一种 Rabbit 抗体,靶向 Galactosidase alpha。Anti-Galactosidase alpha Polyclonal Antibody 可用于 IHC-P,IHC-Fr,IF。
| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 50 μL | ¥ 1,170 | 5日内发货 | |
| 100 μL | ¥ 1,970 | 5日内发货 | |
| 200 μL | ¥ 2,790 | 5日内发货 |
| 产品描述 | Anti-Galactosidase alpha Polyclonal Antibody is a Rabbit antibody targeting Galactosidase alpha. Anti-Galactosidase alpha Polyclonal Antibody can be used in IHC-P,IHC-Fr,IF. |
| Ig Type | IgG |
| 反应种属 | Human,Rat (predicted:Mouse,Dog,Pig,Cow,Rabbit) |
| 应用 | IHC-PIHC-FrIF |
| 推荐剂量 | IHC-P: 1:100-500; IHC-Fr: 1:100-500; IF: 1:100-500 |
| 抗体种类 | Polyclonal |
| 宿主来源 | Rabbit |
| 亚细胞定位 | Lysosome. |
| 构建方式 | Hybridoma Polyclonal Antibody |
| 纯化方式 | Protein A purified |
| 性状 | Liquid |
| 缓冲液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 浓度 | 1mg/ml |
| 研究背景 | Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. 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. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. |
| 免疫原 | KLH conjugated synthetic peptide: human Galactosidase alpha |
| 抗原种属 | Human |
| 基因名称 | GLA |
| 基因ID | |
| 蛋白名称 | alpha-galactosidase A |
| Uniprot ID |
| 分子量 | Theoretical: 45 kDa. |
| 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. |
| 运输方式 | Shipping with blue ice. |
| 存储 | store at low temperature | -20°C for 1 year |