验证数据展示
产品信息
86499-1-PBS targets IDUA as part of a matched antibody pair:
MP02673-1: 86499-1-PBS capture and 86499-2-PBS detection (validated in Sandwich ELISA)
Unconjugated rabbit recombinant monoclonal antibody in PBS only (BSA and azide free) storage buffer at a concentration of 1 mg/mL, ready for conjugation. Created using Proteintech’s proprietary in-house recombinant technology. Recombinant production enables unrivalled batch-to-batch consistency, easy scale-up, and future security of supply.
This conjugation ready format makes antibodies ideal for use in many applications including: ELISAs, multiplex assays requiring matched pairs, mass cytometry, and multiplex imaging applications.Antibody use should be optimized by the end user for each application and assay.
| 经测试应用 | WB, Sandwich ELISA, Indirect ELISA Application Description |
| 经测试反应性 | human, mouse, rat |
| 免疫原 |
CatNo: Ag30658 Product name: Recombinant human IDUA protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 441-555 aa of NM_000203 Sequence: YASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGLCSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHVCARPEKPPGQVTRLR 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Recombinant |
| 产品类型 | Antibody |
| 全称 | iduronidase, alpha-L- |
| 别名 | Alpha-L-iduronidase, EC:3.2.1.76, MPS1 |
| 计算分子量 | 73 kDa |
| 观测分子量 | 73-75 kDa |
| GenBank蛋白编号 | NM_000203 |
| 基因名称 | IDUA |
| Gene ID (NCBI) | 3425 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | P35475 |
| 储存缓冲液 | PBS only, pH 7.3. |
| 储存条件 | Store at -80°C. The product is shipped with ice packs. Upon receipt, store it immediately at -80°C |
背景介绍
Iduronidase (L-iduronidase, alpha-L-iduronidase, laronidase) is an enzyme with the systematic name glycosaminoglycan alpha-L-iduronohydrolase. This enzyme catalyzes the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate. It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal alpha-L-iduronic acid residues of these molecules, degrading them (PMID: 4993544,30407). A deficiency in the IDUA protein is associated with mucopolysaccharidoses (MPS). MPS, a type of lysosomal storage disease, is typed I through VII. In this syndrome, glycosaminoglycans accumulate in the lysosomes and cause substantial disease in many different tissues of the body. IDUA mutations result in the MPS 1 phenotype, which is inherited in an autosomal recessive fashion. The defective alpha-L-iduronidase results in an accumulation of heparan and dermatan sulfate within phagocytes, endothelium, smooth muscle cells, neurons, and fibroblasts. Prenatal diagnosis of this enzyme deficiency is possible (PMID:8242073).
