ARG58730
anti-GAA antibody
anti-GAA antibody for ICC/IF,Western blot and Human,Mouse,Rat
概述
| 产品描述 | Rabbit Polyclonal antibody recognizes GAA |
|---|---|
| 反应物种 | Hu, Ms, Rat |
| 应用 | ICC/IF, WB |
| 宿主 | Rabbit |
| 克隆 | Polyclonal |
| 同位型 | IgG |
| 靶点名称 | GAA |
| 抗原物种 | Human |
| 抗原 | Synthetic peptide within aa. 350-450 of Human GAA (NP_000143.2). |
| 偶联标记 | Un-conjugated |
| 別名 | Lysosomal alpha-glucosidase; LYAG; Aglucosidase alfa; Acid maltase; EC 3.2.1.20 |
应用说明
| 应用建议 |
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| 应用说明 | * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. | ||||||
| 阳性对照 | Mouse thymus | ||||||
| 实际分子量 | 105 kDa |
属性
| 形式 | Liquid |
|---|---|
| 纯化 | Affinity purified. |
| 缓冲液 | PBS (pH 7.3), 0.02% Sodium azide and 50% Glycerol. |
| 抗菌剂 | 0.02% Sodium azide |
| 稳定剂 | 50% Glycerol |
| 存放说明 | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
| 注意事项 | For laboratory research only, not for drug, diagnostic or other use. |
生物信息
| 数据库连接 | |
|---|---|
| 基因名称 | GAA |
| 全名 | glucosidase, alpha; acid |
| 背景介绍 | This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008] |
| 生物功能 | Essential for the degradation of glygogen to glucose in lysosomes. [UniProt] |
| 细胞定位 | Lysosome, Lysosome membrane. [UniProt] |
| 预测分子量 | 105 kDa |
| 翻译后修饰 | The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. [UniProt] |
检测图片 (1) Click the Picture to Zoom In
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ARG58730 anti-GAA antibody WB image
Western blot: 25 µg of Mouse thymus lysate stained with ARG58730 anti-GAA antibody at 1:1000 dilution.
