GBE1 抗体 (AA 79-515)
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北京 101111
Quick Overview for GBE1 抗体 (AA 79-515) (ABIN7876802)
抗原
See all GBE1 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 79-515
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原理
- GBE1 Antibody / 1,4-alpha-glucan branching enzyme 1
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纯化方法
- Immunogen affinity purified
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免疫原
- E.coli-derived human GBE1 recombinant protein (Position: H79-R515) was used as the immunogen for the GBE1 antibody.
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亚型
- IgG
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anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibody
GBE1 适用: 人 WB, IHC, IP, FACS, IHC (p) 宿主: 小鼠 Monoclonal 5B7 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibodyGBE1 适用: 人 WB, IF, IP, FACS 宿主: 小鼠 Monoclonal 4D9 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibodyKD Validated GBE1 适用: 人 WB, FACS 宿主: 小鼠 Monoclonal 24GB13015 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 527-556) antibodyGBE1 适用: 人, 小鼠 WB, IF 宿主: 兔 Polyclonal RB24029 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) antibodyGBE1 适用: 人 WB, IHC, IF, IP, FACS 宿主: 小鼠 Monoclonal 1D11 unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-702) antibodyGBE1 适用: 人 WB, IP 宿主: 兔 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 605-702) antibodyGBE1 适用: 人 WB, ELISA 宿主: 小鼠 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-702) antibodyGBE1 适用: 人 WB 宿主: 小鼠 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 1-300) antibodyGBE1 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal unconjugated
anti-Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1) (AA 534-564), (Middle Region) antibodyGBE1 适用: 人, 小鼠 WB, IF, EIA 宿主: 兔 Polyclonal unconjugated
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应用备注
- Optimal dilution of the GBE1 antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the GBE1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
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别名
- GBE1
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背景
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GBE1 antibody detects 1,4-alpha-glucan branching enzyme 1, an essential enzyme in glycogen biosynthesis responsible for introducing alpha-1,6 glycosidic branches into linear glucan chains. Encoded by the GBE1 gene on chromosome 3p12.3, this enzyme catalyzes the formation of glycogen's highly branched structure, which enhances solubility and allows rapid synthesis and mobilization of glucose. GBE1 is a crucial metabolic regulator in liver, skeletal muscle, and other tissues involved in energy storage and glucose homeostasis.
Structurally, GBE1 is a 702-amino-acid cytosolic enzyme of approximately 80 kilodaltons that belongs to the glycoside hydrolase family 13. It contains a central catalytic domain with the conserved Asp-Glu-Asp catalytic triad and carbohydrate-binding modules that enable glucan chain recognition and rearrangement. By transferring short alpha-1,4-linked oligosaccharide chains to alpha-1,6 positions, GBE1 generates the branch points that define glycogen's compact and accessible configuration.
The GBE1 antibody is widely used in glycogen metabolism, endocrinology, and neuromuscular research to study carbohydrate storage, enzymatic regulation, and metabolic disease mechanisms. Western blot analysis detects an 80 kilodalton band corresponding to GBE1, while immunofluorescence shows diffuse cytoplasmic staining, particularly in hepatocytes and myocytes. This antibody supports investigations of glycogen biosynthesis and the enzymatic defects underlying glycogen storage disorders.
Mutations in GBE1 cause Glycogen Storage Disease Type IV (Andersen disease) and its non-lethal variant, adult polyglucosan body disease (APBD). These disorders are characterized by abnormal glycogen accumulation and progressive neuromuscular or hepatic dysfunction. Reduced GBE1 activity leads to poorly branched glycogen with decreased solubility and impaired mobilization. The GBE1 antibody provides a reliable tool for studying enzyme expression and activity in models of metabolic disease, helping to elucidate molecular mechanisms that govern glycogen structure and energy regulation. NSJ Bioreagents validates this antibody for its applications, ensuring high specificity and reproducibility in metabolic research. -
UniProt
- Q04446
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途径
- Cellular Glucan Metabolic Process
抗原
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