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GAA 抗体 (N-Term)

This anti-GAA antibody is a 兔 多克隆 antibody detecting GAA in WB, IHC 和 IHC (p). Suitable for 人.
产品编号 ABIN656475
发货至: 中国

Quick Overview for GAA 抗体 (N-Term) (ABIN656475)

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

  • 52
  • 8
多克隆

标记

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This GAA antibody is un-conjugated

应用范围

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  • 1
Western Blotting (WB), Immunohistochemistry (IHC), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))

克隆位点

RB18979
  • 抗原表位

    • 15
    • 7
    • 4
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    • 3
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    • 2
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    • 1
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    AA 174-203, N-Term

    纯化方法

    This antibody is purified through a protein A column, followed by peptide affinity purification.

    免疫原

    This GAA antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 174-203 amino acids from the N-terminal region of human GAA.

    亚型

    IgG
  • 应用备注

    WB: 1:2000. WB: 1:1000. WB: 1:1000. WB: 1:1000. IHC-P-Leica: 1:500. IHC-P-Leica: 1:500

    限制

    仅限研究用
  • 状态

    Liquid

    缓冲液

    Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

    储存液

    Sodium azide

    注意事项

    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

    储存条件

    4 °C,-20 °C

    储存方法

    GAA Antibody (N-term) can be refrigerated at 2-8 °C for up to 6 months. For long term storage, place the at -20 °C.

    有效期

    6 months
  • 抗原

    GAA (Glucosidase, Alpha, Acid (GAA))

    别名

    GAA

    背景

    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.

    分子量

    105324

    基因ID

    2548

    NCBI登录号

    NP_000143, NP_001073271, NP_001073272

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
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