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- bs-13254R
- 2025年10月16日
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50ul/100ul/200ul
| 规格： | 50ul | 产品价格： | ￥1180.0 |
|---|---|---|---|
| 规格： | 100ul | 产品价格： | ￥1980.0 |
| 规格： | 200ul | 产品价格： | ￥2800.0 |
| 产品编号 | bs-13254R |
| 英文名称 | GAA Rabbit pAb |
| 中文名称 | 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 | IHC-P=1:100-500, IHC-F=1:100-500, IF=1:200-800 Not yet tested in other applications. |
| 交叉反应 | Mouse (Human, Rat, Dog, Pig, Horse, Sheep) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha |
| 亚型 | IgG |
| 性状 | Liquid |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 理论分子量 | 83/92/98 kDa |
| 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. 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. Contains 1 P-type (trefoil) domain. |
| 功能 | Essential for the degradation of glygogen to glucose in lysosomes. Subcellular Location : Lysosome. Lysosome membrane. |
| 保存条件 | Shipped at 4℃. Store at -20℃ 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 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:200-800} |
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文献和实验[IF={{ 5.23 }}] {Rauniyar et al. Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis. (2015) Mol.Cell.Proteomics. 14:1734-49} {WB} {Human}
= NAL), appears to form upon oxidative cyclization of the nonfluorescent 2:1 lysine-HNE Michael adduct-Schiff base cross-link (Scheme 1). Polyclonal antibody (PAb) to the NAL-HNE fluorophore was raised in rabbit and found to be highly specific
-GCC-AGT-3' M13 (-40) universal forward 5'-GTT-TTC-CCA-GTC-ACG-AC-3' M13/pUC reverse primer 5'-CAG-GAA-ACA-GCT-ATG-ACC-3' T7 primer 5'-TAA-TAC-GAC-TCA-CTA-TAG-GG-3' SP6 primer 5'-ATT-TAG-GTG-ACA-CTA-TAG-3' -16bs 5'-TCG-AGG
) universal forward 5'-GTT-TTC-CCA-GTC-ACG-AC-3' M13/pUC reverse primer 5'-CAG-GAA-ACA-GCT-ATG-ACC-3' T7 primer 5'-TAA-TAC-GAC-TCA-CTA-TAG-GG-3' SP6 primer 5'-ATT-TAG-GTG-ACA-CTA-TAG-3' -16bs 5'-TCG-AGG-TCG-ACG-GTA-TCG-3' +19bs 5'-GCC-GCT
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