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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, ELISA 和 FACS. Suitable for 人.
产品编号 ABIN5775909
发货至: 中国
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Tel +86 (0512) 65829739 传真 +86 (010) 6788 5057

Quick Overview for Hexosaminidase A 抗体 (AA 89-529) (ABIN5775909)

抗原

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

适用

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宿主

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小鼠

克隆类型

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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), ELISA, Flow Cytometry (FACS)

克隆位点

AT20F1
  • 抗原表位

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    AA 89-529

    原理

    Human Hexosaminidase A/HEXA antibody

    纯化方法

    Purified

    免疫原

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

    亚型

    IgG2a lambda
  • 限制

    仅限研究用
  • 状态

    Liquid

    浓度

    1 mg/mL

    缓冲液

    Phosphate-Buffered Saline ( pH 7.4) with 0.02 % Sodium Azide, 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,-80 °C

    储存方法

    Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.
  • 抗原

    Hexosaminidase A (HEXA)

    别名

    Hexosaminidase A/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.

    NCBI登录号

    NP_000511

    途径

    Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
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