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GAMT Protein, Human, Recombinant (His)

Catalog No. TMPJ-01114
Synonyms: 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)
Pack Size Availability Price/USD Quantity
10 μg 5 days $ 116.00
50 μg 5 days $ 352.00
500 μg 5 days $ 1,900.00
1 mg 5 days $ 2,730.00
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Biological Description
Technical Params
Product Properties
Description 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.
Species Human
Expression System E. coli
Tag N,C-6His
Accession Number Q14353
Synonyms Guanidinoacetate N-methyltransferase, TP53I2, PIG2, GAMT
Amino Acid Met1-Gly236
Construction 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.
Protein Purity Greater than 90% as determined by reducing SDS-PAGE. (QC verified)
Molecular Weight 27-32 KDa, reducing conditions
Endotoxin Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test.
Formulation Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 1mM DTT, pH 8.0.
Stability & Storage

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.

Shipping

The product is shipped on dry ice/polar packs. Upon receipt, store it immediately at the temperature listed below.

Research Background 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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