Human Glycine amidinotransferase, mitochondrial (GATM) ELISA Kit | GATM elisa kit
Human Glycine amidinotransferase, mitochondrial (GATM) ELISA Kit
Principle of the Assay: GATM ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-GATM antibody and an GATM-HRP conjugate. The assay sample and buffer are incubated together with GATM-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the GATM concentration since GATM from samples and GATM-HRP conjugate compete for the anti-GATM antibody binding site. Since the number of sites is limited, as more sites are occupied by GATM from the sample, fewer sites are left to bind GATM-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The GATM concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]
Uniprot Description
GATM: Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis. Defects in GATM are the cause of arginine:glycine amidinotransferase deficiency (AGAT deficiency). AGAT deficiency is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain. Belongs to the amidinotransferase family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - arginine and proline; EC 2.1.4.1; Transferase; Mitochondrial; Amino Acid Metabolism - glycine, serine and threonine
Chromosomal Location of Human Ortholog: 15q21.1
Cellular Component: mitochondrial inner membrane; mitochondrial intermembrane space
Molecular Function: hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amidines; glycine amidinotransferase activity
Biological Process: response to mercury ion; response to peptide hormone stimulus; creatine biosynthetic process; tissue regeneration; response to oxidative stress; creatine metabolic process; response to nutrient
Disease: Cerebral Creatine Deficiency Syndrome 3
Research Articles on GATM
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Product Notes
The Human GATM gatm (Catalog #AAA7247595) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7247595 ELISA Kit recognizes Human GATM. It is sometimes possible for the material contained within the vial of "Glycine amidinotransferase, mitochondrial (GATM), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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