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GLDH enzyme :: Glutamate Dehydrogenase Enzyme

Scan QR to view Datasheet Catalog #    MBS682068 GLDH enzyme
Unit / Price
600 Units  /  $285 +1 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   

Glutamate Dehydrogenase (GLDH), Enzyme

★Popular Item★
 Also Known As   

Glutamate Dehydrogenase (Yeast) (GLDH)

 Product Synonym Names    Glutamate dehydrogenase (Yeast); GLDH (yeast)
 Product Gene Name   

GLDH enzyme

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Chromosome Location    Chromosome: 10; NC_000010.10 (88809959..88854776, complement). Location: 10q23.3
 OMIM    138130
 3D Structure    ModBase 3D Structure for P00367
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 Form/Format    Lyophilized Powder
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 Specifications    L-Glutamate: NADP+ oxidoreductase (deaminating) (EC 1.4.1.4) from yeast
 Contaminant    (as GlDH activity = 100%)
Glucose-6-phosphate dehydrogenase < 0.1 %
Phosphogluconate dehydrogenase < 0.5 %
Glutamate dehydrogenase (NAD+) < 0.1 %
Glutathione reductase < 0.1 %
NADPH oxidase < 0.01 %
 Reaction Equation    L-Glutamate + H2O + NADP+ = 2-oxoglutarate + NH3 + NADPH
 Specific Activity    > 10 U/mg protein
(for reduction of alpha-ketoglutarate to L-glutamate at 25 degree C and pH 7.6)
 State    Ammonium sulfate free, lyophilized powder of highly purified enzyme
One mg of powder contains the following
Protein 700 +/- 200 ug
K-PO4 300 +/- 300 ug
 Preparation    Lyophilized
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 Preparation and Storage    Lyophilized at -20 degree C.
 Other Notes    Small volumes of GLDH enzyme 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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 Product Categories/Family for GLDH enzyme    Enzymes
NCBI/Uniprot data below describe general gene information for GLDH. It may not necessarily be applicable to this product.
 NCBI GI #    31799
 NCBI GeneID    2746
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 UniProt Primary Accession #    P00367 [Other Products]
 UniProt Secondary Accession #    Q5TBU3 [Other Products]
 UniProt Related Accession #    P00367; Q14400; Q53GW3; Q59FQ4; Q9UQV0 [Other Products]
 Molecular Weight    61,398 Da
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 NCBI Official Full Name    glutamate dehydrogenase
 NCBI Official Synonym Full Names    glutamate dehydrogenase 1
 NCBI Official Symbol    GLUD1 [Similar Products]
 NCBI Official Synonym Symbols   
GDH; GDH1; GLUD; MGC132003
[Similar Products]
 NCBI Protein Information    glutamate dehydrogenase 1, mitochondrial; GDH 1; OTTHUMP00000020018; glutamate dehydrogenase (NAD(P)+)
 UniProt Protein Name    Glutamate dehydrogenase 1, mitochondrial
 Protein Family    Glutamate dehydrogenase
 UniProt Gene Name    GLUD1 [Similar Products]
 UniProt Synonym Gene Names    GLUD [Similar Products]
 UniProt Entry Name    DHE3_HUMAN
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 NCBI Summary for GLDH    This gene encodes glutamate dehydrogenase protein; a mitochondrial matrix enzyme that catalyzes the oxidative deamination of glutamate to alpha-ketoglutarate and ammonia. This enzyme has an important role in regulating amino acid induced insulin secretion and activating mutations in this gene are a common cause of congenital hyperinsulinism. This enzyme is allosterically activated by ADP and inhibited by GTP and ATP. The related glutamate dehydrogenase 2 gene on the human X-chromosome originated from this gene via retrotransposition and encodes a soluble form of glutamate dehydrogenase. Multiple pseudogenes of this gene are present in humans.
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 UniProt Comments for GLDH    Function: May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate

Catalytic activity: L-glutamate + H2O + NAD(P)+ = 2-oxoglutarate + NH3 + NAD(P)H.

Enzyme regulation: Subject to allosteric regulation. Activated by ADP. Inhibited by GTP and ATP. ADP can occupy the NADH binding site and activate the enzyme.

Subunit structure: Homohexamer.

Subcellular location: Mitochondrion matrix.

Involvement in disease: Defects in GLUD1 are the cause of familial hyperinsulinemic hypoglycemia type 6 (HHF6) [

MIM:606762]; also known as hyperinsulinism-hyperammonemia syndrome (HHS). Familial hyperinsulinemic hypoglycemia [

MIM:256450], also referred to as congenital hyperinsulinism, nesidioblastosis, or persistent hyperinsulinemic hypoglycemia of infancy (PPHI), is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. In HHF6 elevated oxidation rate of glutamate to alpha-ketoglutarate stimulates insulin secretion in the pancreatic beta cells, while they impair detoxification of ammonium in the liver. Ref.22 Ref.23 Ref.24 Ref.25

Sequence similarities: Belongs to the Glu/Leu/Phe/Val dehydrogenases family.
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 Research Articles on GLDH    1. In Chinese, R269H, S445L mutation of the GLUD1 leads to congenital hyperinsulinism.
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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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Pathways associated with GLDH enzymeDiseases associated with GLDH enzyme
 Products by Pathway  Pathway Diagram
 Alanine, Aspartate And Glutamate Metabolism Pathway antibodies  Alanine, Aspartate And Glutamate Metabolism Pathway Diagram
 Alanine, Aspartate And Glutamate Metabolism Pathway antibodies  Alanine, Aspartate And Glutamate Metabolism Pathway Diagram
 Amino Acid Synthesis And Interconversion (transamination) Pathway antibodies  Amino Acid Synthesis And Interconversion (transamination) 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
 D-Glutamine And D-glutamate Metabolism Pathway antibodies  D-Glutamine And D-glutamate Metabolism Pathway Diagram
 D-Glutamine And D-glutamate Metabolism Pathway antibodies  D-Glutamine And D-glutamate Metabolism Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Nitrogen Metabolism Pathway antibodies  Nitrogen Metabolism Pathway Diagram
 Disease Name  Pubmed Publications
 Hyperinsulinism Antibodies  >30 publications with GLDH and Hyperinsulinism
 Hyperammonemia Antibodies  >15 publications with GLDH and Hyperammonemia
 Nervous System Diseases Antibodies  >4 publications with GLDH and Nervous System Diseases
 Diabetes Mellitus Antibodies  >2 publications with GLDH and Diabetes Mellitus
 Neoplasms Antibodies  >2 publications with GLDH and Neoplasms
 Obesity Antibodies  >1 publications with GLDH and Obesity
 Pancreatic Neoplasms Antibodies  >1 publications with GLDH and Pancreatic Neoplasms
 Leukemia Antibodies  >1 publications with GLDH and Leukemia
 Inflammation Antibodies  >1 publications with GLDH and Inflammation
 Liver Diseases Antibodies  >1 publications with GLDH and Liver Diseases
Organs/Tissues associated with GLDH enzyme
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >24 publications with GLDH and Brain
 Pancreas Antibodies  >13 publications with GLDH and Pancreas
 Nerve Antibodies  >10 publications with GLDH and Nerve
 Blood Antibodies  >10 publications with GLDH and Blood
 Testis Antibodies  >4 publications with GLDH and Testis
 Liver Antibodies  >4 publications with GLDH and Liver
 Placenta Antibodies  >2 publications with GLDH and Placenta
 Bone Antibodies  >1 publications with GLDH and Bone
 Eye Antibodies  >1 publications with GLDH and Eye
 Spinal Cord Antibodies  >1 publications with GLDH and Spinal Cord
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