Glycine amidinotransferase, mitochondrial (GATM), ELISA Kit

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C.

We suggest pre-experimenting with neat (undiluted) samples, 1:2 or 1:4 dilutions. Please avoid diluting your samples more than 1:10 as it would exceed the dilution limit set for this kit. If the expected concentration of the target is beyond the detection range of the kit, please contact our technical support team

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of GATM elisa kit 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.

MBS7235033 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the Glycine amidinotransferase, mitochondrial (GATM) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing GATM. The ELISA analytical biochemical technique of the MBS7235033 kit is based on GATM antibody-GATM antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect GATM antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, GATM. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

44,943 Da

glycine amidinotransferase (L-arginine:glycine amidinotransferase)

glycine amidinotransferase, mitochondrial; transamidinase

Glycine amidinotransferase, mitochondrial

AGAT  [Similar Products]

GATM_HUMAN

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]

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 - glycine, serine and threonine; Mitochondrial; Transferase; EC 2.1.4.1; Amino Acid Metabolism - arginine and proline

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

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