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GBE1 抗体 (AA 101-200) (Cy7)

This anti-GBE1 antibody (ABIN1707697) is a Rabbit Polyclonal antibody detecting GBE1 in WB, IF (cc), IF (p). Suitable for Human.
产品编号 ABIN1707697
发货至: 中国

Quick Overview for GBE1 抗体 (AA 101-200) (Cy7) (ABIN1707697)

抗原

See all GBE1 抗体
GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

适用

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宿主

  • 41
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克隆类型

  • 44
  • 6
多克隆

标记

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This GBE1 antibody is conjugated to Cy7

应用范围

  • 41
  • 18
  • 12
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  • 1
Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
  • 抗原表位

    • 14
    • 7
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    • 1
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    • 1
    AA 101-200

    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
  • 应用备注

    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200

    限制

    仅限研究用
  • 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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

    有效期

    12 months
  • 抗原

    GBE1 (Glucan (1,4-alpha-), Branching Enzyme 1 (GBE1))

    别名

    GBE1

    背景

    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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