验证数据展示
产品信息
29354-1-PBS targets AASS in WB, IHC, IP, Indirect ELISA applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | WB, IHC, IP, Indirect ELISA Application Description |
| 经测试反应性 | human, mouse, rat |
| 免疫原 |
CatNo: Ag29520 Product name: Recombinant human AASS protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 33-146 aa of NM_005763 Sequence: VNAWERRAPLAPKHIKGITNLGYKVLIQPSNRRAIHDKDYVKAGGILQEDISEACLILGVKRPPEEKLMSRKTYAFFSHTIKAQEANMGLLDEILKQEIRLIDYEKMVDHRGVR 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | aminoadipate-semialdehyde synthase |
| 别名 | LKR, LKR/SDH, LKRSDH, LOR, LORSDH |
| 计算分子量 | 102 kDa |
| 观测分子量 | 100-110 kDa |
| GenBank蛋白编号 | NM_005763 |
| 基因名称 | AASS |
| Gene ID (NCBI) | 10157 |
| RRID | AB_3742311 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | Q9UDR5 |
| 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
Aminoadipate-semialdehyde synthase (AASS) is a bifunctional enzyme catalyzing the first two steps of lysine degradation via the saccharopine pathway. It possesses both lysine-ketoglutarate reductase and saccharopine dehydrogenase activities. Mutations in the AASS gene cause familial hyperlysinemia, a rare autosomal recessive disorder characterized by elevated plasma lysine levels, though clinical manifestations are typically benign. AASS dysfunction disrupts mitochondrial energy metabolism and amino acid homeostasis. Beyond its metabolic role, emerging evidence suggests AASS may influence cellular redox status and autophagy regulation, positioning it as a potential therapeutic target for metabolic disorders and lysine-related pathologies (PMID: 41158987; 10775527).





