Overview
| 别名 | Iduronate 2-sulfatase; Alpha-L-iduronate sulfate sulfatase; Idursulfase) [Cleaved into: Iduronate 2-sulfatase 42 kDa chain; Iduronate 2-sulfatase 14 kDa chain] |
| 基因名 | IDS |
| UniProt ID | P22304 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 6I8-S5-P7 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 61 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200-1:250 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Iduronate 2-sulfatase (IDS) is a lysosomal enzyme that catalyzes the hydrolysis of sulfate esters in glycosaminoglycans, specifically heparan sulfate and dermatan sulfate. The 550-amino acid protein is encoded by the IDS gene on the X chromosome and undergoes post-translational processing to form a mature enzyme. IDS contains eight potential N-linked glycosylation sites, which are important for its stability and lysosomal targeting. The enzyme's crystal structure reveals insights into its catalytic mechanism and the impact of pathogenic mutations. Deficiency of IDS leads to mucopolysaccharidosis type II (Hunter syndrome), an X-linked lysosomal storage disorder characterized by the accumulation of partially degraded glycosaminoglycans. This accumulation results in progressive damage to various tissues and organs, with clinical manifestations ranging from mild to severe forms of the disease. |
检测原理