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GAMT Protein, Human, Recombinant (His)

GAMT Protein, Human, Recombinant (His)

产品编号 TMPJ-01114
别名: Guanidinoacetate N-methyltransferase, TP53I2, PIG2, GAMT

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)
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
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产品目录号及名称: GAMT Protein, Human, Recombinant (His) (TMPJ-01114)
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产品性质
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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Keywords

GAMT Protein, Human, Recombinant (His) PIG-2 Guanidinoacetate N-methyltransferase PIG 2 TP53I2 PIG2 GAMT recombinant recombinant-proteins proteins protein

 

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