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- bs-13254R-Cy7
- 2025年09月30日
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100ul
| 产品编号 | bs-13254R-Cy7 |
| 英文名称 | GAA Rabbit pAb, Cy7 conjugated |
| 中文名称 | Cy7标记的α葡萄糖苷酶/溶酶体α-葡糖苷酶抗体 |
| 英文别名 | 70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; alpha glucosidase; GAA; Glucosidase alpha acid(Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase. |
| 产品应用 | IF=1:200-800 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism Signal Transduction > Metabolism > Energy Metabolism |
| 亚细胞定位 | Lysosome. Lysosome membrane. |
| 翻译后修饰 | The different forms of acid glucosidase are obtained by proteolytic processing. _x000D_ Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. |
| 相似性 | Belongs to the glycosyl hydrolase 31 family. _x000D_ Contains 1 P-type (trefoil) domain. |
| 功能 | Essential for the degradation of glygogen to glucose in lysosomes._x000D_ Subcellular Location : Lysosome. Lysosome membrane. |
| 保存条件 | Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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]. |
| 应用 | 推荐稀释比例 |
| {IF} | {1:200-800} |
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GAA Rabbit pAb, Cy7 conjugated(bs-13254R-Cy7)-100ul
￥2980
