HEXB 抗体
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北京 101111
Quick Overview for HEXB 抗体 (ABIN7881035)
抗原
See all HEXB 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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原理
- Hexosaminidase B Antibody / HEXB
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纯化方法
- Protein A/G affinity
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免疫原
- A recombinant fragment of the human protein was used as the immunogen for the Hexosaminidase B antibody.
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亚型
- IgG2, kappa
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anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 122-311) antibody
HEXB 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 294-435) antibodyHEXB 适用: 小鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 315-456) antibodyHEXB 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 101-217) antibodyHEXB 适用: 小鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (C-Term) antibodyHEXB 适用: 人, 小鼠, 大鼠 WB, IHC, ELISA, ICC, IF 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 381-556) antibodyHEXB 适用: 人 WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (C-Term) antibodyHEXB 适用: 人 WB, IHC, ELISA 宿主: 兔 Polyclonal unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) antibodyMicroarray verified HEXB 适用: 人, 小鼠 IHC 宿主: 小鼠 Monoclonal MAB220161 unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) antibodyMicroarray verified HEXB 适用: 人, 小鼠 IHC 宿主: 小鼠 Monoclonal MAB461847 unconjugated
anti-Hexosaminidase B (Beta Polypeptide) (HEXB) (AA 100-216) antibodyHEXB 适用: 大鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
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应用备注
- Optimal dilution of the Hexosaminidase B antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 0.2 mg/mL
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缓冲液
- 0.2 mg/mL in 1X PBS with 0.1 mg/mL BSA (US sourced), 0.05 % sodium azide
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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
- -20 °C
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储存方法
- Aliquot the Hexosaminidase B antibody and store frozen at -20oC or colder. Avoid repeated freeze-thaw cycles.
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- HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))
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别名
- Hexosaminidase B
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背景
- Hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a hexosaminidase B (HEXB), also designated beta-hexosaminidase B, is a tetramer of two beta-A and two beta-B chains and is found in the lysosomes of cells. Sandhoff disease (SD), also known as GM2-gangliosidosis type II, is caused by mutations in the HEXB gene that affect the beta subunit. These mutations disrupt the activity of HEXB and HEXA, which prevents the breakdown of GM2 ganglioside, a fatty material found in the brain, therby rendering both the HEXA and HEXB enzymes deficient. SD is a rare autosomal recessive disorder characterized by an accumulation of GM2 ganglioside, which causes progressive destruction of the central nervous system. Sandhoff disease is similar to Tay-Sachs disease, which is caused by mutations in the HEXA gene, although SD is more severe.
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UniProt
- P07686
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途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
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