Overview
| 别名 | Thiamine pyrophosphokinase 1; hTPK1; Placental protein 20; PP20; Thiamin pyrophosphokinase 1 |
| 基因名 | TPK1 |
| UniProt ID | Q9H3S4 |
| 反应种属 | Human,Mouse |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 9E2-R7-R3 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 27 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 | Thiamin pyrophosphokinase 1 (TPK1) is a homodimeric enzyme that plays a vital role in energy metabolism by catalyzing the conversion of thiamine (vitamin B1) into thiamine pyrophosphate (TPP). Utilizing ATP or UTP as phosphate donors, TPK1 facilitates the production of TPP, an essential cofactor for key enzymes in glycolysis, the Krebs cycle, and acetyl-CoA formation. The enzyme features a structural architecture comprising an alpha/beta-domain and a beta-sandwich domain, with the active site located at the dimer interface. TPK1 is highly expressed in metabolically active tissues such as the kidney and liver. Clinically, defects in the TPK1 gene are the primary cause of thiamine metabolism dysfunction syndrome-5 (TMDS5), a rare autosomal recessive disorder characterized by severe lactic acidosis, progressive Leigh-like encephalopathy, and early-onset sensorineural hearing loss. |
检测原理