GATM, Polyclonal Antibody

AGAT; AT

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

Polyclonal

IgG

Rabbit

Antigen affinity purification

Store at -20 degree C. Avoid freeze / thaw cycles

Manufactured in an ISO 9001:2015 Certified Laboratory.

Small volumes of anti-GATM antibody 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.

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:500-1:5000
IHC: 1:50-1:200

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

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; creatine biosynthetic process; response to peptide hormone stimulus; tissue regeneration; response to oxidative stress; creatine metabolic process; response to nutrient

Disease: Cerebral Creatine Deficiency Syndrome 3

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