HEXB 抗体
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北京 101111
Quick Overview for HEXB 抗体 (ABIN7861437)
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
- HEXB Antibody
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特异性
- Hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a hexosaminidase B (HEXB), also designated ≤-hexosaminidase B, is a tetramer of two ≤-A and two ≤-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 ≤ 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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纯化方法
- 200ug/ml of Ab purified from Bioreactor Concentrate by Protein A/G.
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免疫原
- Recombinant fragment human HEXB protein (exact sequence is proprietary)
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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 working dilution should be determined by the investigator.
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限制
- 仅限研究用
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浓度
- 1.0 mg/mL
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缓冲液
- Prepared in 10 mM PBS
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储存液
- Azide free
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储存条件
- -20 °C,-80 °C
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储存方法
- Antibody with azide - store at 2 to 8°C. Antibody without azide - store at -20 to -80°C.Antibody is stable for 24 months. Non-hazardous. No MSDS required.
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- HEXB (Hexosaminidase B (Beta Polypeptide) (HEXB))
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别名
- HEXB
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背景
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Beta N acetylhexosaminidase, Cervical cancer proto oncogene 7 protein, ENC 1AS, Epididymis luminal protein 248, HCC7, HEL 248, Hexosaminidase B (beta polypeptide), Hexosaminidase B, N-acetyl-beta-glucosaminidase subunit beta
Human kidney or lung.
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分子量
- 63kDa
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基因ID
- 3074, 69293
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途径
- Sensory Perception of Sound, Glycosaminoglycan Metabolic Process
抗原
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