Hexosaminidase A 抗体
Quick Overview for Hexosaminidase A 抗体 (ABIN1105521)
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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特异性
- Recognizes Beta-hexosaminidase alpha / HEXA
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交叉反应 (详细)
- Species reactivity (tested):Human.
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纯化方法
- Purified
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亚型
- IgG2b
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应用备注
- Optimal working dilution should be determined by the investigator.
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1.0 mg/mL
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缓冲液
- PBS, 0.05 % Sodium Azide, 0.5 % protein stabilizer
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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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- Hexosaminidase A (HEXA)
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别名
- beta-Hexosaminidase alpha / HEXA
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背景
- This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).Synonyms: Beta-N-acetylhexosaminidase subunit alpha, N-acetyl-beta-glucosaminidase subunit alpha
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分子量
- 60.7 kDa
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基因ID
- 3073
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途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
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