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GAA 抗体

This anti-GAA antibody is a 兔 单克隆 antibody detecting GAA in WB, IHC (p) 和 FACS. Suitable for 人.
产品编号 ABIN6989688
发货至: 中国

Quick Overview for GAA 抗体 (ABIN6989688)

抗原

See all GAA 抗体
GAA (Glucosidase, Alpha, Acid (GAA))

适用

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宿主

  • 54
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克隆类型

  • 53
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单克隆

标记

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This GAA antibody is un-conjugated

应用范围

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Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Flow Cytometry (FACS)

克隆位点

5H49
  • 交叉反应

    纯化方法

    Purified by Protein A.

    免疫原

    Synthetic peptide within Human GAA.

    亚型

    IgG
  • 应用备注

    WB 1:300-5000
    FCM 1:20-100
    IHC-P 1:200-400

    限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 μg/μL

    缓冲液

    Aqueous buffered solution containing 1xTBS ( pH 7.4), 1 % BSA, 40 %Glycerol and 0.05 % Sodium Azide.

    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
  • 抗原

    GAA (Glucosidase, Alpha, Acid (GAA))

    别名

    GAA

    背景

    Synonyms: 70 kDa lysosomal alpha-glucosidase antibody, Acid alpha glucosidase antibody, Acid maltase antibody, Aglucosidase alfa antibody, Alpha glucosidase antibody, GAA antibody, Glucosidase alpha acid (Pompe disease glycogen storage disease type II) antibody, Glucosidase alpha acid antibody, Glucosidase alpha antibody, LYAG antibody, LYAG_HUMAN antibody, Lysosomal alpha glucosidase antibody

    Background: This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe's disease, which is an autosomal recessive disorder with a broad clinical spectrum. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008].

    基因ID

    2548

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
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