| 货号 | MAB2449-SP |
| 别名 | Alpha-L-iduronate sulfate sulfatase; EC 3.1.6.13; iduronate 2-sulfatase 14 kDa chain; iduronate 2-sulfatase 42 kDa chain; iduronate 2-sulfatase; idursulfase; MPS2; S; SIDS |
| 反应种属 | Human |
| 应用 | Western Blot(1 µg/mL) |
| 目标/特异性 | Detects human Iduronate 2-Sulfatase/IDS in direct ELISAs and Western blots. In direct ELISAs and Western blots, this antibody does not cross‑react with recombinant mouse Iduronate 2-Sulfatase/IDS. |
| 使用方法 | Western Blot: 1 µg/mL |
| 来源 | Reconstitute at 0.5 mg/mL in sterile PBS. |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 3423 (Human); 15931 (Mouse) |
| 纯化方式 | Protein A or G purified from hybridoma culture supernatant |
| 免疫原 | Mouse myeloma cell line NS0-derived recombinant human Iduronate 2-Sulfatase/IDS Ser26-Pro550 Accession # P22304 |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Reconstitute at 0.5 mg/mL in sterile PBS. |
| 背景 | As a member of the sulfatase family, IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and dermatan sulfate (2, 3). It hydrolyzes the 2-sulfate group of the L-iduronate 2-sulfate units of the GAG. The IDS deficiency results in mucopolysaccharidosis II (MPS II or Hunter syndrome), an X-linked inborn error leading to lysosomal accumulation of the GAG and its excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe. The deduced amino acid sequence of human IDS consists of a signal peptide (residues 1 - 25), a pro peptide (residues 26 - 33) and a mature chain (residues 34 - 550) that may be further processed into the 42 kDa chain (residues 34 - 455) and the 14 kDa chain (residues 456 - 550) (1). Recombinant human IDS corresponds to the single chain and has sulfatase activity described above. |
| 运输条件 | Blue Ice |
| 存放说明 | 4℃ |
| 参考文献 |
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