Overview
| 别名 | Phosphoacetylglucosamine mutase; PAGM; Acetylglucosamine phosphomutase; N-acetylglucosamine-phosphate mutase; Phosphoglucomutase-3; PGM 3 |
| 基因名 | PGM3 |
| UniProt ID | O95394 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 1M5-V5-M5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 59 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:250 |
| 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 | Phosphoglucomutase 3 (PGM3) is a cytosolic enzyme that catalyzes the reversible conversion of N-acetylglucosamine-6-phosphate to N-acetylglucosamine-1-phosphate. This reaction is a fundamental step in the biosynthesis of UDP-GlcNAc, a critical precursor for protein N-glycosylation and the hexosamine biosynthetic pathway. PGM3 possesses a characteristic heart-shaped architecture with four structural domains and a conserved catalytic phosphoserine loop. Biallelic loss-of-function or hypomorphic mutations in PGM3 result in an autosomal recessive congenital disorder of glycosylation characterized by severe immunodeficiency, elevated IgE, recurrent infections, and neurodevelopmental delay. These clinical manifestations reflect the essential role of PGM3-dependent glycosylation in immune cell function and skeletal development. |
检测原理