| 货号 | AF2449-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) Immunoprecipitation(25 µg/mL) |
| 目标/特异性 | Detects human IDS in direct ELISAs and Western blots. In direct ELISAs, approximately 50% cross-reactivity with recombinant mouse IDS is observed. |
| 使用方法 | Western Blot: 1 µg/mL Immunoprecipitation: 25 µg/mL |
| 来源 | Reconstitute at 0.2 mg/mL in sterile PBS. |
| 产品组分 |
| 供应商 | R&D Systems |
| Entrez Gene IDs | 3423 (Human); 15931 (Mouse) |
| 应用文献 | |
| R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions. Localized donor cells in brain of a Hunter disease patient after cord blood stem cell transplantation. | |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | Mouse myeloma cell line NS0-derived recombinant human IDS Ser26-Pro550 Accession # P22304 |
| 生物活性 | Human |
| 标记 | Unconjugated |
| 溶解方法 | Reconstitute at 0.2 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 |
|
Detection of Human Iduronate 2‑Sulfatase/IDS by Western Blot. Western blot shows lysates of human peripheral blood mononuclear cells (PBMCs). PVDF membrane was probed with 1 µg/mL of Goat Anti-Human Iduronate 2‑Sulfatase/IDS Antigen Affinity-purified Polyclonal Antibody (Catalog # AF2449) followed by HRP-conjugated Anti-Goat IgG Secondary Antibody (Catalog # HAF017). A specific band was detected for Iduronate 2‑Sulfatase/IDS at approximately 42 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1. |
