Overview
| 别名 | O-phosphoseryl-tRNA(Sec) selenium transferase; Liver-pancreas antigen; LP; SLA-p35; SLA/LP autoantigen; Selenocysteine synthase; Sec synthase; Selenocysteinyl-tRNA(Sec) synthase; Sep-tRNA:Sec-tRNA synthase; SepSecS; Soluble liver antigen; SLA; UGA suppressor tRNA-associated protein; tRNA(Ser/Sec)-associated antigenic protein |
| 基因名 | SEPSECS |
| UniProt ID | Q9HD40 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8Q6-D1-O4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 55 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:4000 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Sep (O-phosphoserine) tRNA:Sec (selenocysteine) tRNA synthase (SEPSECS) is a homotetrameric enzyme that catalyzes the final, rate-limiting step in the biosynthesis of selenocysteine. It facilitates the conversion of O-phosphoseryl-tRNA(Sec) to selenocysteinyl-tRNA(Sec) using selenophosphate and pyridoxal phosphate (PLP) as essential cofactors. The enzyme forms a complex with tRNA(Sec), employing a unique mammalian C-terminal helix to regulate stoichiometry and stabilize the complex. SEPSECS is also recognized as the soluble liver antigen/liver pancreas (SLA/LP) autoantigen. Clinically, it is a diagnostic marker for autoimmune hepatitis, and pathogenic variants in the SEPSECS gene cause progressive cerebello-cerebral atrophy type 2 (PCCA2), a severe neurodevelopmental disorder characterized by microcephaly and cerebellar atrophy. |
检测原理