| 货号 | BAF4119 |
| 别名 | IDA; IDUA |
| 反应种属 | Human |
| 应用 | Western Blot(0.1 µg/mL) |
| 目标/特异性 | Detects human alpha ‑L‑Iduronidase/IDUA in Western blots. |
| 使用方法 | Western Blot: 0.1 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 | 3425 (Human); 15932 (Mouse); 360904 (Rat) |
| 纯化方式 | Antigen Affinity-purified |
| 免疫原 | Mouse myeloma cell line NS0-derived recombinant human alpha ‑L‑Iduronidase/IDUA Ala26-Pro653 (Ala26Thr) Accession # AAA81589 |
| 生物活性 | Human |
| 标记 | Biotin |
| 溶解方法 | Reconstitute at 0.2 mg/mL in sterile PBS. |
| 背景 | alpha -L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non‑reducing terminal alpha -L-iduronic acid residues in GAGs including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits (2). |
| 运输条件 | Blue Ice |
| 存放说明 | -20℃ |
| 参考文献 |
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