| 别名 | SGSH;HSS;N-sulphoglucosamine sulphohydrolase ;Sulfoglucosamine sulfamidase;Sulphamidase; |
| 基因名称 | SGSH |
| 蛋白名称 | N-sulphoglucosamine sulphohydrolase;Sulfoglucosamine sulfamidase;Sulphamidase; |
| 推荐应用 | |
| 反应物种 | Human |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | 6448 |
| Human Swissprot No. | P51688 |
| 特异性 | endogenous |
| 稀释度 | WB 1:1000-1:2500； |
| 参考分子量 | MW(Observed):55kD |
| 预测分子量 | MW(Calculated):55kD |
| 运输及保存条件 | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 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. |
| 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 | Protein A |
| Clonality | Monoclonal |
| 修饰类型 | Unmodified |
| 细胞定位 | Lysosome. |
| 组织表达 | Liver, Kidney, Brain, Hepatocellular carcinoma, Neuroblastoma, Lung cancer |
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