GBE1 抗体 (AA 101-200) (Cy7)
Quick Overview for GBE1 抗体 (AA 101-200) (Cy7) (ABIN1707697)
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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                                            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
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                                            限制
- 仅限研究用
 
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    - 
                                            状态
- Liquid
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                                            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.
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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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