GBE1 抗体 (AA 101-200) (Cy3)
Quick Overview for GBE1 抗体 (AA 101-200) (Cy3) (ABIN1702117)
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 101-200
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预测反应
- Human,Mouse,Rat,Rabbit
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纯化方法
- Purified by Protein A.
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免疫原
- KLH conjugated synthetic peptide derived from human GBE1
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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) 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 1-300) antibodyGBE1 适用: 人 WB, ELISA, IHC 宿主: 兔 Polyclonal 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 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) (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
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 -
限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 μg/μL
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缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
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有效期
- 12 months
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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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Synonyms: 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.
Background: 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.
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基因ID
- 2632
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途径
- Cellular Glucan Metabolic Process
抗原
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