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GAMT recombinant protein :: Guanidinoacetate N-Methyltransferase Recombinant Protein

Scan QR to view Datasheet Catalog #    MBS143485 GAMT recombinant protein
Unit / Price
0.005 mg  /  $140 +1 FREE 8GB USB
0.025 mg  /  $205 +1 FREE 8GB USB
1 mg  /  $2,235 +2 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Guanidinoacetate N-Methyltransferase (GAMT), Recombinant Protein

 Also Known As   

Recombinant Human Guanidinoacetate N-Methyltransferase

 Product Synonym Names    GAMT Human; Guanidinoacetate N-Methyltransferase Human Recombinant; PIG2; TP53I2; GAMT; Guanidinoacetate N-methyltransferase
 Product Gene Name   

GAMT recombinant protein

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Sequence    MGSSHHH HHH 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
 OMIM    601240
 3D Structure    ModBase 3D Structure for Q14353
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 Host    E Coli
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 Purity/Purification    Greater than 95.0% as determined by SDS-PAGE.
 Form/Format    The GAMT protein solution contains 20mM Tris-HCl, pH-8, 1mM DTT and 10% Glycerol.
Sterile filtered colorless solution.
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 Preparation and Storage    Store at 4 degree C if entire vial will be used within 2-4 weeks. Store, frozen at -20 degree C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA). Avoid multiple freeze-thaw cycles.
 Other Notes    Small volumes of GAMT recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for GAMT recombinant protein

   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 20 amino acid His-Tag at N-terminus and purified by conventional chromatography techniques.

Introduction: GAMT is a methyltransferase that transfers guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor. Defects GAMT gene result in neurologic syndromes and muscular hypotonia, probably due to creatine deficiency and accumulation of guanidinoacetate in the brain of affected individuals. GAMT take parts in the two-step synthesis of creatine from the protein building blocks glycine, arginine, and methionine. GAMT takes part in supplying the energy for muscle contraction, and is in addition a significant player in nervous system functioning. GAMT is active in the liver, pancreas, and kidne.
 Product Categories/Family for GAMT recombinant protein    ENZYMES; Enzymes; Transferase
NCBI/Uniprot data below describe general gene information for GAMT. It may not necessarily be applicable to this product.
 NCBI GI #    4503909
 NCBI GeneID    2593
 NCBI Accession #    NP_000147.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_000156.5 [Other Products]
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 UniProt Primary Accession #    Q14353 [Other Products]
 UniProt Secondary Accession #    Q53Y34; Q8WVJ1; A8K0A0 [Other Products]
 UniProt Related Accession #    Q14353 [Other Products]
 Molecular Weight    29,377 Da
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 NCBI Official Full Name    guanidinoacetate N-methyltransferase isoform a
 NCBI Official Synonym Full Names    guanidinoacetate N-methyltransferase
 NCBI Official Symbol    GAMT [Similar Products]
 NCBI Official Synonym Symbols   
PIG2; CCDS2; TP53I2; HEL-S-20
[Similar Products]
 NCBI Protein Information    guanidinoacetate N-methyltransferase; epididymis secretory protein Li 20
 UniProt Protein Name    Guanidinoacetate N-methyltransferase
 Protein Family    Guanidinoacetate N-methyltransferase
 UniProt Gene Name    GAMT [Similar Products]
 UniProt Entry Name    GAMT_HUMAN
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 NCBI Summary for GAMT    The protein encoded by this gene is a methyltransferase that converts guanidoacetate to creatine, using S-adenosylmethionine as the methyl donor. Defects in this gene have been implicated in neurologic syndromes and muscular hypotonia, probably due to creatine deficiency and accumulation of guanidinoacetate in the brain of affected individuals. Two transcript variants encoding different isoforms have been described for this gene. Pseudogenes of this gene are found on chromosomes 2 and 13. [provided by RefSeq, Feb 2012]
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 UniProt Comments for GAMT    GAMT: Defects in GAMT are the cause of guanidinoacetate methyltransferase deficiency (GAMT deficiency). GAMT deficiency 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 (GAA) in brain and body fluids. Belongs to the class I-like SAM-binding methyltransferase superfamily. RMT2 methyltransferase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Methyltransferase; EC 2.1.1.2; Amino Acid Metabolism - glycine, serine and threonine; Contractile; Amino Acid Metabolism - arginine and proline

Chromosomal Location of Human Ortholog: 19p13.3

Cellular Component: cytosol

Molecular Function: methyltransferase activity; guanidinoacetate N-methyltransferase activity

Biological Process: methylation; creatine biosynthetic process; organ morphogenesis; muscle contraction; regulation of multicellular organism growth; spermatogenesis; creatine metabolic process

Disease: Cerebral Creatine Deficiency Syndrome 2
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 Research Articles on GAMT    1. Study reports six novel pathogenic mutations in GAMT gene in patients with Guanidinoacetate methyltransferase deficiency.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with GAMT recombinant proteinPathways associated with GAMT recombinant protein
 Reference Product  PubMed Publications
 GATM recombinant protein  >4 publications with GAMT and GATM
 CKB recombinant protein  >1 publications with GAMT and CKB
 Products by Pathway  Pathway Diagram
 Arginine And Proline Metabolism Pathway antibodies  Arginine And Proline Metabolism Pathway Diagram
 Arginine And Proline Metabolism Pathway antibodies  Arginine And Proline Metabolism Pathway Diagram
 Creatine Metabolism Pathway antibodies  Creatine Metabolism Pathway Diagram
 Creatine Pathway antibodies  Creatine Pathway Diagram
 Creatine Pathway antibodies  Creatine Pathway Diagram
 Glycine, Serine And Threonine Metabolism Pathway antibodies  Glycine, Serine And Threonine Metabolism Pathway Diagram
 Glycine, Serine And Threonine Metabolism Pathway antibodies  Glycine, Serine And Threonine Metabolism Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
Diseases associated with GAMT recombinant proteinOrgans/Tissues associated with GAMT recombinant protein
 Disease Name  Pubmed Publications
 Nervous System Diseases Antibodies  >56 publications with GAMT and Nervous System Diseases
 Brain Diseases Antibodies  >42 publications with GAMT and Brain Diseases
 Guanidinoacetate methyltransferase deficiency Antibodies  >35 publications with GAMT and Guanidinoacetate methyltransferase deficiency
 Seizures Antibodies  >23 publications with GAMT and Seizures
 Intellectual Disability Antibodies  >21 publications with GAMT and Intellectual Disability
 Amino Acid Metabolism, Inborn Errors Antibodies  >18 publications with GAMT and Amino Acid Metabolism, Inborn Errors
 Heart Diseases Antibodies  >6 publications with GAMT and Heart Diseases
 Liver Diseases Antibodies  >6 publications with GAMT and Liver Diseases
 Muscular Diseases Antibodies  >5 publications with GAMT and Muscular Diseases
 Learning Disorders Antibodies  >5 publications with GAMT and Learning Disorders
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >80 publications with GAMT and Brain
 Blood Antibodies  >58 publications with GAMT and Blood
 Liver Antibodies  >43 publications with GAMT and Liver
 Muscle Antibodies  >26 publications with GAMT and Muscle
 Heart Antibodies  >15 publications with GAMT and Heart
 Kidney Antibodies  >14 publications with GAMT and Kidney
 Pancreas Antibodies  >8 publications with GAMT and Pancreas
 Embryonic Tissue Antibodies  >5 publications with GAMT and Embryonic Tissue
 Testis Antibodies  >3 publications with GAMT and Testis
 Eye Antibodies  >3 publications with GAMT and Eye
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