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- bs-13479R-AP
- 2025年12月01日
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100ul
| 产品编号 | bs-13479R-AP |
| 英文名称 | GNS Rabbit pAb, AP conjugated |
| 中文名称 | AP标记的氨基葡萄糖6-硫酸酯酶抗体 |
| 英文别名 | 2610016K11Rik; AU042285; C87209; G6S; Glucosamine(N-acetyl) 6 sulfatase; Glucosamine 6 sulfatase; Glucosamine-6-sulfatase; GNS; GNS_HUMAN; MGC21274; N acetylglucosamine 6 sulfatase [Precursor]; N-acetylglucosamine-6-sulfatase; N28088. |
| 产品应用 | IHC-P=1:100-500, IHC-F=1:100-500, ELISA=1:500-5000 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human GNS/Glucosamine 6 sulfatase |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cell Biology > Proteolysis / Ubiquitin > Proteolytic enzymes > Other proteases Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism Signal Transduction > Metabolism > Amino Acids Tags & Cell Markers > Subcellular Markers > Organelles > Lysosome |
| 亚细胞定位 | Lysosome. |
| 翻译后修饰 | The form A (78 kDa) is processed by internal peptidase cleavage to a 32 kDa N-terminal species (form B) and a 48 kDa C-terminal species. The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteine residue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity. |
| 相似性 | Belongs to the sulfatase family. |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 背景资料 | GNS is a 552 amino acid lysosomal enzyme that hydrolyzes the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate and heparan sulfate. A member of the sulfatase family, GNS assists in the catabolism of heparin, and binds calcium as a cofactor. GNS deficiency results in an autosomal recessive lysosomal storage disorder known as mucopolysaccharidosis type IIID (Sanfilippo D syndrome), which is characterized by mild somatic disease and severe degeneration of the central nervous system. Subject to post-translational internal peptidase cleavage, GNS is encoded by a gene mapping to human chromosome 12q14.2 and mouse chromosome 10 D2. |
| 应用 | 推荐稀释比例 |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {ELISA} | {1:500-5000} |
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GNS Rabbit pAb, AP conjugated(bs-13479R-AP)-100ul
￥2980
