SGSH 抗体 (AA 273-502)
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- 抗原 See all SGSH 抗体
- SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
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抗原表位
- AA 273-502
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适用
- 人
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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 SGSH antibody is un-conjugated
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应用范围
- Immunohistochemistry (IHC), ELISA
- 交叉反应
- 人
- 纯化方法
- Antigen Affinity Purified
- 免疫原
- Recombinant Human N-sulphoglucosamine sulphohydrolase protein (273-502AA)
- 亚型
- IgG
- Top Product
- Discover our top product SGSH Primary Antibody
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- 应用备注
- Recommended dilution: IHC:1:20-1:200,
- 限制
- 仅限研究用
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- 状态
- Liquid
- 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,-80 °C
- 储存方法
- Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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- 抗原
- SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
- 别名
- SGSH (SGSH 产品)
- 别名
- HSS antibody, MPS3A antibody, SFMD antibody, 4632406A19Rik antibody, N-sulfoglucosamine sulfohydrolase antibody, N-sulfoglucosamine sulfohydrolase (sulfamidase) antibody, SGSH antibody, Sgsh antibody
- 背景
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Background: SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62 kDa precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A.
Aliases: Heparan sulfate sulfatase antibody, Heparan sulphate sulphatase antibody, HSS antibody, MPS 3A antibody, MPS3 A antibody, MPS3A antibody, Mucopolysaccharidosis type IIIA antibody, N sulfoglucosamine sulfohydrolase (sulfamidase) antibody, N sulfoglucosamine sulfohydrolase antibody, N-sulphoglucosamine sulphohydrolase antibody, SFMD antibody, SGSH antibody, SPHM_HUMAN antibody, Sulfoglucosamine sulfamidase antibody, Sulphamidase antibody, Sulphoglucosamine sulphamidase antibody
- UniProt
- P51688
- 途径
- Glycosaminoglycan Metabolic Process
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