SGSH 抗体 (AA 301-388) (AbBy Fluor® 594)
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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 301-388
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适用
- 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 SGSH antibody is conjugated to AbBy Fluor® 594
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应用范围
- Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
- 交叉反应
- 大鼠
- 预测反应
- Human,Mouse,Dog
- 纯化方法
- Purified by Protein A.
- 免疫原
- KLH conjugated synthetic peptide derived from human Sulphamidase
- 亚型
- IgG
- Top Product
- Discover our top product SGSH Primary Antibody
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- 应用备注
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IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200 - 限制
- 仅限研究用
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- 状态
- Liquid
- 浓度
- 1 μg/μL
- 缓冲液
- Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
- 储存液
- ProClin
- 注意事项
- This product contains ProClin: 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. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- 有效期
- 12 months
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- 抗原
- SGSH (N-Sulfoglucosamine Sulfohydrolase (SGSH))
- 别名
- SGSH/Sulphamidase (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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Synonyms: HSS, SFMD, MPS3A, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH
Background: Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.
- 基因ID
- 6448
- UniProt
- P51688
- 途径
- Glycosaminoglycan Metabolic Process
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