| 产品名称 | 
 Recombinant Human GAMT (N, C-6His)  | 
| 英文名称 | 
 Guanidinoacetate N-methyltransferase/GAMT  | 
| 纯度 | 
 Greater than 95% as determined by reducing SDS-PAGE  | 
| 内毒素 | 
 <1 EU/µg as determined by LAL test.  | 
| 蛋白构建 | 
 Recombinant Human Guanidinoacetate N-methyltransferase is produced by our E.coli expression system and the target gene encoding Met1-Gly236 is expressed with a 6His tag at the N-terminus, 6His tag at the C-terminus.  | 
| Accession | 
 Q14353  | 
| 表达宿主 | 
 E.coli  | 
| 种属 | 
 Human  | 
| 预测分子量 | 
 29.5 KDa  | 
| 制剂 | 
 Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 1mM DTT, pH 8.0.  | 
| 运输方式 | 
 The product is shipped on dry ice/polar packs.Upon receipt, store it immediately at the temperature listed below.  | 
| 稳定性&储存 | 
 Store at ≤-70°C, stable for 6 months after receipt.Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles.  | 
| 复溶 | 
| 分子别名 | 
| Guanidinoacetate N-methyltransferase; GAMT; PIG2; TP53I2 | 
| 背景介绍 | 
| GAMT is a methyltransferase which belongs to the class I-like SAM-binding methyltransferase superfamily. It contains one RMT2 (arginine N-methyltransferase 2-like) domain and is expressed in liver. GAMT converts guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor. Defects in GAMT are the cause of guanidinoacetate methyltransferase deficiency, which is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, intractable seizures and movement disturbances, severe depletion of creatine/phosphocreatine in the brain, and accumulation of guanidinoacetic acid in brain and body fluids. | 
注意事项
本司产品仅用于科研,不用于临床诊断和治疗