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Anti-IDUA Polyclonal Antibody 是一种 Rabbit 抗体,靶向 IDUA。Anti-IDUA Polyclonal Antibody 可用于 WB,ELISA。
Anti-IDUA Polyclonal Antibody 是一种 Rabbit 抗体,靶向 IDUA。Anti-IDUA Polyclonal Antibody 可用于 WB,ELISA。
| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 50 μL | ¥ 1,175 | 5日内发货 | |
| 100 μL | ¥ 1,965 | 5日内发货 |
| 产品描述 | Anti-IDUA Polyclonal Antibody is a Rabbit antibody targeting IDUA. Anti-IDUA Polyclonal Antibody can be used in WB,ELISA. |
| Ig Type | IgG |
| 反应种属 | Human,Mouse |
| 应用 | WBELISA |
| 推荐剂量 | WB: 1:500-2000; ELISA: 1:5000-10000 |
| 抗体种类 | Polyclonal |
| 宿主来源 | Rabbit |
| 亚细胞定位 | Lysosome. |
| 组织特异性 | Ubiquitous. |
| 构建方式 | Hybridoma Polyclonal Antibody |
| 纯化方式 | Protein A purified |
| 性状 | Liquid |
| 缓冲液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 浓度 | 1mg/ml |
| 研究背景 | This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). [provided by RefSeq, Jul 2008]. |
| 免疫原 | KLH conjugated synthetic peptide: human IDUA |
| 抗原种属 | Human |
| 基因名称 | IDUA |
| 基因ID | |
| 蛋白名称 | alpha-L-iduronidase |
| Uniprot ID | |
| 功能 | IDUA is an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Defects in IDUA are the cause of mucopolysaccharidosis type 1H (MPS1H) also known as Hurler syndrome, mucopolysaccharidosis type 1H/S (MPS1H/S) also known as Hurler-Scheie syndrome and mucopolysaccharidosis type 1S (MPS1S) also known as Scheie syndrome. MPS1S is a mild form whilst MPS1H is a severe form of this rare lysosomal storage disease characterized by progressive physical deterioration with urinary excretion of dermatan sulfate and heparan sulfate. |
| 分子量 | Theoretical: 70 kDa. |
| 储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| 运输方式 | Shipping with blue ice. |
| 存储 | store at low temperature | -20°C for 1 year |