产品概述
| 产品名称(Product Name) | Galactosidase alpha (4R19) Rabbit Monoclonal Antibody |
| 描述(Description) | Rabbit Monoclonal Antibody |
| 宿主(Host) | Rabbit |
| 应用(Application) | WB |
| 种属反应性(Reactivity) | Human |
产品性能
| 偶联物(Conjugation) | Unconjugated |
| 修饰(Modification) | Unmodified |
| 同种型(Isotype) | IgG |
| 克隆(Clonality) | Monoclonal |
| 形式(Form) | Liquid |
| 存放说明(Storage) | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 储存溶液(Buffer) | Supplied in 50mM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% New type preservative N and 0.05% BSA. |
| 纯化方式(Purification) | Affinity purification |
免疫原
| 免疫原信息(Immunogen Information) | 0 |
| 基因名(Gene Name) | GLA |
| 别名(Alternative Names) | Alpha gal A; GALA; Galactosidase, alpha; GLA; Melibiase; |
| 基因ID(Gene ID) | 2717 |
| 蛋白ID(SwissProt ID) | P06280 |
产品应用
| 稀释比(Dilution Ratio) | WB: 1:1000 |
| 蛋白分子量(Molecular Weight) | 49kDa |
研究背景
Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. Catalyzes the hydrolysis of glycosphingolipids and participates in their degradation in the lysosome.
研究领域