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- bs-13300R
- 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-13300R |
| 英文名称 | GBE1 Rabbit pAb |
| 中文名称 | 分支酶GBE1抗体 |
| 英文别名 | 1, 4 alpha glucan branching enzyme; 4-alpha-glucan-branching enzyme; amylo(1, 4 to 1, 6) transglucosidase; amylo(1, 4 to 1, 6) transglycosylase; Andersen disease; Brancher enzyme; GBE 1; GBE; GBE1; gGlucan(1, 4 alpha ), branching enzyme 1; GLGB_HUMAN; Glucan(1, 4 alpha) branching enzyme; Glycogen branching enzyme; Glycogen storage disease type IV; Glycogen-branching enzyme; OTTHUMP00000213788; OTTHUMP00000213833. |
| 产品应用 | WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, ICC/IF=1:100-500, IF=1:100-500, ELISA=1:5000-10000 Not yet tested in other applications. |
| 交叉反应 | (Human, Mouse, Rat, Rabbit) |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human GBE1 |
| 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 | 80 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 | Cancer > Cancer Metabolism > Metabolic signaling pathway > Metabolism of carbohydrates Metabolism > Pathways and Processes > Metabolic signaling pathways > Carbohydrate metabolism Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism Signal Transduction > Metabolism > Energy Metabolism |
| 组织特异性 | Highest levels found in liver and muscle. |
| 相似性 | Belongs to the glycosyl hydrolase 13 family. |
| 功能 | Required for sufficient glycogen accumulation. The alpha 1-6 branches of glycogen play an important role in increasing the solubility of the molecule and, consequently, in reducing the osmotic pressure within cells. |
| 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 | GBE1 is a 702 amino acid protein that is expressed at high levels in muscle and liver and is involved in glycogen biosynthesis. Existing as a monomer, GBE1 catalyzes the transfer of alpha-1,4-linked glucosyl units from the outer end of a glycogen chain to an alpha-1,6 position on a neiGHBoring glycogen chain and, via this catalytic activity, plays an essential role in glycogen accumulation. Defects in the gene encoding GBE1 are the cause of glycogen storage disease type 4 (GSD4) and adult polyglucosan body disease (APBD), the first of which is a metabolic disorder that is associated with the accumulation of polysaccharides and is characterized by liver disease during childhood. Unlike GSD4, APBD is a late-onset disorder that affects the central and peripheral nervous systems and is characterized by cognitive impairment, pyramidal tetraparesis and peripheral neuropathy. |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {ICC/IF} | {1:100-500} |
| {IF} | {1:100-500} |
| {ELISA} | {1:5000-10000} |
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