| 货号 | AF4119-SP |
| 别名 | IDA; IDUA |
| 反应种属 | Human |
| 应用 | Western Blot(0.1 µg/mL) Immunoprecipitation(25 µg/mL) Immunocytochemistry(5-15 µg/mL) |
| 目标/特异性 | Detects human alpha ‑L‑Iduronidase/IDUA in direct ELISAs and Western blots. |
| 使用方法 | Western Blot: 0.1 µg/mL Immunoprecipitation: 25 µg/mL Immunocytochemistry: 5-15 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 | Unconjugated |
| 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 | 4℃ |
| 参考文献 |
|
alpha ‑L‑Iduronidase/IDUA in HepG2 Human Cell Line. alpha ‑L‑Iduronidase/IDUA was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Sheep Anti-Human alpha ‑L‑Iduronidase/IDUA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF4119) at 15 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Sheep IgG Secondary Antibody (red; Catalog # NL010) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. View our protocol for Fluorescent ICC Staining of Cells on Coverslips. |
