Overview
| 别名 | Alanyl-tRNA editing protein Aarsd1; Alanyl-tRNA synthetase domain-containing protein 1 |
| 基因名 | AARSD1 |
| UniProt ID | Q9BTE6 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8H9-N3-C1 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 45 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000 |
| 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 | Alanyl-tRNA synthetase domain containing 1 (AARSD1) is a nuclear protein belonging to the class-II aminoacyl-tRNA synthetase family. It functions in trans to edit mischarged tRNA(Ala) by providing Ser-tRNA(Ala) deacylase activity, which removes incorrectly attached serine residues to ensure translational fidelity and accurate protein synthesis. The protein possesses nucleic acid binding capability and may utilize zinc as a cofactor. AARSD1 is ubiquitously expressed across human tissues. Clinical relevance is established through its association with rare autosomal recessive neurological disorders, specifically Distal Hereditary Motor Neuronopathy Type 1, characterized by progressive distal muscle weakness and atrophy resulting from lower motor neuron degeneration. |
检测原理