GBE1 抗体 (AA 101-200) (Biotin)
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- 抗原 See all GBE1 抗体
- GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
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抗原表位
- AA 101-200
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适用
- 人
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This GBE1 antibody is conjugated to Biotin
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应用范围
- Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
- 预测反应
- Human,Mouse,Rat,Rabbit
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human GBE1
- 亚型
- IgG
- Top Product
- Discover our top product GBE1 Primary Antibody
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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 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, IP 宿主: 兔 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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WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- 储存条件
- -20 °C
- 储存方法
- Store at -20°C for 12 months.
- 有效期
- 12 months
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- 抗原
- GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))
- 别名
- GBE1 (GBE1 产品)
- 别名
- glgBII antibody, APBD antibody, GBE antibody, GSD4 antibody, 2310045H19Rik antibody, 2810426P10Rik antibody, D16Ertd536e antibody, 1,4-alpha-glucan branching protein GlgB antibody, 1,4-alpha-glucan branching protein antibody, glycogen branching protein antibody, glycogen branching enzyme antibody, 1,4-alpha-glucan-branching protein antibody, glycogen branching enzyme; BE; 1,4-alpha-D-glucan:1,4-alpha-D-glucan 6-glucosyl-transferase antibody, 1,4-alpha-glucan branching enzyme 1 antibody, glucan (1,4-alpha-), branching enzyme 1 antibody, glgB antibody, ECs4277 antibody, SCO7332 antibody, Synpcc7942_1085 antibody, AZC_4116 antibody, Sros_8169 antibody, Despr_0975 antibody, GBE1 antibody, Gbe1 antibody
- 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.
- 基因ID
- 2632
- 途径
- Cellular Glucan Metabolic Process
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