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GAA 抗体 (AA 174-203)

This 兔 多克隆 antibody specifically detects GAA in WB 和 IHC (p). It exhibits reactivity toward 人.
产品编号 ABIN7871285
发货至: 中国
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Quick Overview for GAA 抗体 (AA 174-203) (ABIN7871285)

抗原

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

适用

  • 51
  • 24
  • 22
  • 2
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1

宿主

  • 50
  • 5

克隆类型

  • 47
  • 8
多克隆

标记

  • 35
  • 4
  • 3
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
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This GAA antibody is un-conjugated

应用范围

  • 48
  • 18
  • 13
  • 13
  • 13
  • 9
  • 8
  • 8
  • 5
  • 3
  • 2
  • 1
Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
  • 抗原表位

    • 15
    • 6
    • 4
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 174-203

    原理

    GAA Antibody / Glucosidase alpha acid

    纯化方法

    Antigen affinity purified

    免疫原

    A portion of amino acids 174-203 from the human protein were used as the immunogen for the GAA antibody.

    亚型

    Ig Fraction
  • 应用备注

    The stated application concentrations are suggested starting points. Titration of the GAA antibody may be required due to differences in protocols and secondary/substrate sensitivity.

    限制

    仅限研究用
  • 状态

    Liquid

    缓冲液

    In 1X PBS, pH 7.4, with 0.09 % 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

    -20 °C

    储存方法

    Aliquot the GAA antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
  • 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.

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
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