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.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
10 μg | ¥ 740 | 5日内发货 | ||
50 μg | ¥ 2,240 | 5日内发货 | ||
500 μg | ¥ 12,100 | 5日内发货 | ||
1 mg | ¥ 17,400 | 5日内发货 |
产品描述 | 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. |
种属 | Human |
表达系统 | E. coli |
标签 | N, C-6xHis |
蛋白编号 | Q14353 |
别名 | Guanidinoacetate N-methyltransferase, TP53I2, PIG2, GAMT |
氨基酸序列 | Met1-Gly236 |
蛋白构建 | Met1-Gly236 |
蛋白纯度 | Greater than 90% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 27-32 KDa (reducing condition) |
内毒素 | < 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
缓冲液 | Supplied as a 0.2 μm filtered solution of 20 mM Tris-HCl, 1 mM DTT, pH 8.0. |
存储 |
Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots. |
运输方式 |
In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
研究背景 | 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. |
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GAMT Protein, Human, Recombinant (His) PIG-2 Guanidinoacetate N-methyltransferase PIG 2 TP53I2 PIG2 GAMT recombinant recombinant-proteins proteins protein