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GAA 抗体 (AA 494-527)

This anti-GAA antibody is a 兔 多克隆 antibody detecting GAA in WB 和 IHC (p). Suitable for 人 和 大鼠.
产品编号 ABIN5647392
发货至: 中国

Quick Overview for GAA 抗体 (AA 494-527) (ABIN5647392)

抗原

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

适用

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人, 大鼠

宿主

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克隆类型

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多克隆

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))
  • 抗原表位

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    AA 494-527

    纯化方法

    Antigen affinity purified

    免疫原

    Amino acids 494-527 (TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR-human) were used as the immunogen for the GAA antibody.

    亚型

    IgG
  • 应用备注

    Optimal dilution of the GAA antibody should be determined by the researcher.\. Western blot: 0.5-1 μg/mL,Immunohistochemistry (FFPE): 1-2 μg/mL

    限制

    仅限研究用
  • 缓冲液

    0.5 mg/mL if reconstituted with 0.2 mL sterile DI water

    储存条件

    -20 °C

    储存方法

    After reconstitution, the GAA antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
  • 抗原

    GAA (Glucosidase, Alpha, Acid (GAA))

    别名

    GAA / Glucosidase alpha acid

    背景

    Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. 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. Alternative splicing results in multiple transcript variants.

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
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