Hexosaminidase A 抗体
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- 抗原 See all Hexosaminidase A (HEXA) 抗体
- Hexosaminidase A (HEXA)
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适用
- 人, 小鼠, 大鼠
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宿主
- 兔
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克隆类型
- 多克隆
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标记
- This Hexosaminidase A antibody is un-conjugated
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应用范围
- Immunohistochemistry (IHC)
- 产品特性
- Polyclonal Antibody
- 纯化方法
- Affinity purification
- 免疫原
- Recombinant fusion protein of human HEXA (NP_000511.2).
- 亚型
- IgG
- Top Product
- Discover our top product HEXA Primary Antibody
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- 应用备注
- IHC 1:50-1:200
- 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 mg/mL
- 缓冲液
- PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3
- 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
- -20 °C
- 储存方法
- Store at -20°C. Avoid freeze / thaw cycles.
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- 抗原
- Hexosaminidase A (HEXA)
- 别名
- HEXA (HEXA 产品)
- 别名
- TSD antibody, fc04h11 antibody, wu:fc04h11 antibody, wu:fv09f06 antibody, si:dkey-35i22.2 antibody, HEXA antibody, Hex-1 antibody, zgc:112084 antibody, hexosaminidase subunit alpha antibody, hexosaminidase B (beta polypeptide) antibody, CUGBP Elav-like family member 6 antibody, hexosaminidase A antibody, hexosaminidase A (alpha polypeptide) antibody, beta-hexosaminidase subunit alpha-like antibody, HEXA antibody, hexb antibody, CELF6 antibody, Hexa antibody, hexa antibody, LOC100228061 antibody
- 背景
- This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.
- 基因ID
- 3073
- UniProt
- P06865
- 途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
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