验证数据展示
产品信息
86131-2-PBS targets ADSL in WB, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | WB, Indirect ELISA Application Description |
| 经测试反应性 | human, mouse, rat |
| 免疫原 | ADSL fusion protein Ag7332 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 | adenylosuccinate lyase |
| 别名 | AMPS, ASASE, ASL, EC:4.3.2.2 |
| 计算分子量 | 55 kDa |
| 观测分子量 | 55 kDa |
| GenBank蛋白编号 | BC000253 |
| 基因名称 | ADSL |
| Gene ID (NCBI) | 158 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | P30566 |
| 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
ADSL(adenylosuccinate lyase) is also named as AMPS, ASase, ASL and belongs to the lyase 1 family. It is an enzyme involved in 2 pathways of purine nucleotide metabolism and catalyzes cleavage of succinyl groups to yield fumarate (PMID:18524658). Defects in ADSL are the cause of adenylosuccinase deficiency (ADSL deficiency). In humans, mutations in ADSL lead to an inborn error of metabolism originally characterized by developmental delay, often with autistic features (PMID:20884265). The ADSL enzymatic activity is reduced in lymphocytes and red blood cells of the patient with severe psychomotor retardation (PMID:9545543). It has 2 isoforms produced by alternative splicing.