| 别名 | N-sulphoglucosamine sulphohydrolase;Sulfoglucosamine sulfamidase;Sulphamidase; |
| 基因名称 | Endogenous |
| 蛋白名称 | SGSH;HSS |
| 推荐应用 | |
| 反应物种 | SGSH |
| 存储缓冲液 | WB 1:1000-5000;IHC 1:100-300;ICC/IF 1:100-300 |
| Human Gene Link | SGSH;HSS;N-sulphoglucosamine sulphohydrolase ;Sulfoglucosamine sulfamidase;Sulphamidase; |
| Human Swissprot No. | 6448 |
| Mouse Gene ID | P51688 |
| 特异性 | Monoclonal |
| 稀释度 | Unmodified |
| 预测分子量 | WB,IHC,IF,ICC |
| 运输及保存条件 | Protein A |
| 宿主 | 55kD |
| 同种型 | Rabbit |
| 背景介绍 | This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined. [provided by RefSeq, Jun 2017] |
| 细胞定位 | Lysosome. |
| 功能 | Catalyzes a step in lysosomal heparan sulfate degradation. {ECO:0000269|PubMed:15146460, ECO:0000269|PubMed:24816101, ECO:0000269|PubMed:7493035}. |
| 纯化 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Clonality | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 修饰类型 | IgG,Kappa |
| 细胞定位 | Lysosome. |
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