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Hexosaminidase A 抗体 (AA 89-529)

This anti-Hexosaminidase A antibody is a 小鼠 单克隆 antibody detecting Hexosaminidase A in WB, FACS 和 EIA. Suitable for 人.
产品编号 ABIN5540052
发货至: 中国

Quick Overview for Hexosaminidase A 抗体 (AA 89-529) (ABIN5540052)

抗原

See all Hexosaminidase A (HEXA) 抗体
Hexosaminidase A (HEXA)

适用

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宿主

  • 53
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小鼠

克隆类型

  • 55
  • 12
单克隆

标记

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This Hexosaminidase A antibody is un-conjugated

应用范围

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Western Blotting (WB), Flow Cytometry (FACS), Enzyme Immunoassay (EIA)

克隆位点

AT20F1
  • 抗原表位

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    AA 89-529

    特异性

    This antibody detects HEXA at aa 89-529.

    纯化方法

    Protein-A affinity chromatography

    免疫原

    Recombinant human HEXA (89-529aa) purified from E. coli

    亚型

    IgG2a
  • 应用备注

    ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.

    限制

    仅限研究用
  • 状态

    Liquid

    缓冲液

    PBS, pH 7.4 containing 0.02 % Sodium Azide and 10 % Glycerol

    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

    Store undiluted at 2-8°C for up to two weeks or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing. Shelf life: one year from despatch.

    有效期

    12 months
  • 抗原

    Hexosaminidase A (HEXA)

    别名

    beta-hexosaminidase alpha,hexa

    背景

    HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS.

    UniProt

    P06865

    途径

    Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
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