Overview
| 别名 | Phosphopantothenate--cysteine ligase; Phosphopantothenoylcysteine synthetase; PPC synthetase |
| 基因名 | PPCS |
| UniProt ID | Q9HAB8 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2b |
| 克隆号 | 7U4-F6-L8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 34 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:100-1:200 |
| 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 | Phosphopantothenoylcysteine synthetase (PPCS) is a dimeric enzyme that catalyzes the second step of the coenzyme A (CoA) biosynthetic pathway. It facilitates the ATP-dependent conjugation of L-cysteine to 4'-phosphopantothenate to form 4'-phosphopantothenoylcysteine. Unlike bacterial orthologs that utilize CTP, human PPCS specifically employs ATP to form a 4'-phosphantothenoyl-adenylate intermediate before the nucleophilic attack by cysteine. This 15.6 kDa protein is essential for maintaining cellular CoA levels, which are critical for numerous metabolic reactions. Clinically, mutations in the PPCS gene are established causes of congenital myasthenic syndrome 2C, a rare neuromuscular disorder characterized by impaired synaptic transmission due to systemic coenzyme A deficiency. |
检测原理