Hexosaminidase A 抗体 (AA 89-529)
Quick Overview for Hexosaminidase A 抗体 (AA 89-529) (ABIN5540052)
抗原
See all Hexosaminidase A (HEXA) 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- AA 89-529
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特异性
- This antibody detects HEXA at aa 89-529.
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纯化方法
- Protein-A affinity chromatography
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免疫原
- Recombinant human HEXA (89-529aa) purified from E. coli
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亚型
- IgG2a
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应用备注
- ELISA. Western blot: Recommended starting dilution is 1:3000. Flow cytometry.
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- PBS, pH 7.4 containing 0.02 % Sodium Azide and 10 % Glycerol
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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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.
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有效期
- 12 months
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- Hexosaminidase A (HEXA)
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别名
- beta-hexosaminidase alpha,hexa
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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.
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UniProt
- P06865
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途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
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