GATM, Monoclonal Antibody

Monoclonal GATM; Anti-GATM; Glycine Amidinotransferase; L-Arginine:Glycine Amidinotransferase; AGAT; AT

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

Monoclonal

IgG1

1C9

Mouse

GATM antibody was purified by affinity chromatography.

Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.

500 ug-1 mg/ml (lot specific)

Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

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.

Mouse monoclonal GATM antibody

Proteases, Inhibitors, & Enzymes

Flow Cytometry (FC/FACS), Immunofluorescence (IF), Immunohistochemistry (IHC), Western Blot (WB)

IF: 1:100
FC: 1:100
IHC: 1:150
WB: 1:500-2000

Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant GATM protein (Right) or empty vector (Left) detected with GATM antibody

Immunohistochemical analysis of GATM protein in paraffin embedded Human liver tissue using GATM antibody

Immunofluorescent staining of COS7 cells transiently transfected with recombinant GATM protein using GATM antibody

44,943 Da

glycine amidinotransferase (L-arginine:glycine amidinotransferase)

glycine amidinotransferase, mitochondrial

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

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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