GAMT Human
Guanidinoacetate N-Methyltransferase Human Recombinant
Cat. No.
BT4292
Source
Escherichia Coli.
Synonyms
PIG2, TP53I2, GAMT, Guanidinoacetate N-methyltransferase.
Appearance
Sterile filtered colorless solution.
Purity
Greater than 95.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description
Recombinant Human GAMT produced in E.Coli is a single, non-glycosylated polypeptide chain containing 256 amino acids (1-236 a.a) and having a molecular mass of 28.4 kDa. GAMT is fused to a 20 amino acids His-Tag at N-terminus and purified by conventional chromatography techniques.
Product Specs
Introduction
GAMT is an enzyme that plays a crucial role in creatine synthesis. It catalyzes the transfer of a methyl group from S-adenosylmethionine to guanidinoacetate, resulting in the formation of creatine. Creatine is essential for energy production and storage in muscles and the brain. Defects in the GAMT gene disrupt creatine synthesis, leading to neurological disorders characterized by muscle weakness and developmental delays. These conditions are thought to arise from creatine deficiency and the buildup of guanidinoacetate in the brain. GAMT participates in a two-step process that generates creatine from the amino acids glycine, arginine, and methionine. This enzyme is primarily active in the liver, pancreas, and kidneys.
Description
Recombinant Human GAMT is a laboratory-produced protein based on the human GAMT enzyme. It is manufactured using E. coli bacteria and consists of a single polypeptide chain containing 256 amino acids. This non-glycosylated protein has a molecular weight of 28.4 kDa. For purification and detection purposes, a 20 amino acid His-Tag is attached to the N-terminus of the protein.
Physical Appearance
Clear and colorless solution that has been sterilized by filtration.
Formulation
This GAMT protein solution is prepared in a buffer consisting of 20mM Tris-HCl at pH 8, 1mM DTT (dithiothreitol), and 10% glycerol.
Stability
For short-term storage (2-4 weeks), the GAMT protein should be kept at 4°C. For longer storage, it is recommended to freeze the protein at -20°C. To ensure stability during long-term storage, consider adding a carrier protein such as HSA or BSA (0.1% concentration). Avoid repeated freezing and thawing of the protein solution.
Purity
The purity of this GAMT protein is greater than 95%, as determined by SDS-PAGE analysis.
Synonyms
PIG2, TP53I2, GAMT, Guanidinoacetate N-methyltransferase.
Source
Escherichia Coli.
Amino Acid Sequence
MGSSHHHHHH SSGLVPRGSH MSAPSATPIF APGENCSPAW GAAPAAYDAA DTHLRILGKP VMERWETPYM HALAAAASSK GGRVLEVGFG MAIAASKVQE APIDEHWIIE CNDGVFQRLR DWAPRQTHKV IPLKGLWEDV APTLPDGHFD GILYDTYPLS EETWHTHQFN FIKNHAFRLL KPGGVLTYCN LTSWGELMKS KYSDITIMFE ETQVPALLEA GFRRENIRTE VMALVPPADC RYYAFPQMIT PLVTKG.
Product Science Overview
Introduction
Structure and Function
GAMT belongs to the family of transferases, specifically those transferring one-carbon groups known as methyltransferases. The enzyme’s systematic name is S-adenosyl-L-methionine:N-guanidinoacetate methyltransferase. The reaction it catalyzes can be summarized as follows :
This reaction is vital for the production of creatine, an essential molecule for energy storage and supply in muscle and brain tissues .
Biological Importance
Clinical Significance
Deficiencies in GAMT activity can lead to a condition known as Cerebral Creatine Deficiency Syndrome (CCDS), which is characterized by neurological symptoms such as developmental delay, intellectual disability, and seizures. This condition results from the accumulation of guanidinoacetate and a deficiency of creatine in the brain .
Recombinant GAMT
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