货号 | AF2486-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 | Mouse |
应用 | Western Blot(0.1 µg/mL) Immunoprecipitation(25 µg/mL) |
目标/特异性 | Detects mouse Iduronate 2‑Sulfatase/IDS in direct ELISAs and Western blots. In direct ELISAs and Western blots, approximately 40% cross‑reactivity with recombinant human IDS is observed. |
使用方法 | Western Blot: 0.1 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) |
纯化方式 | Antigen Affinity-purified |
免疫原 | Mouse myeloma cell line NS0-derived recombinant mouse Iduronate 2‑Sulfatase/IDS Thr36-Pro552 Accession # Q08890 |
生物活性 | Mouse |
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, Iduronate 2-Sulfatase encoded by the IDS gene is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and dermatan sulfate (1, 2). 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 their excretion in urine. MPS II has a wide spectrum of clinical manifestations ranging from mild to severe. The mouse IDS has sulfatase activity. In addition, recombinant human IDS is also available (Catalog # 2449-SU). |
运输条件 | Blue Ice |
存放说明 | 4℃ |
参考文献 |
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