GATM, Polyclonal Antibody

Anti -GATM, NT (GATM, AGAT, Glycine amidinotransferase, mitochondrial, L-arginine:glycine amidinotransferase, Transamidinase)

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

Chromosome: 15; NC_000015.9 (45653322..45670980, complement). Location: 15q21.1

Polyclonal

IgG

Rabbit

Human

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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.

Antibodies; Abs to Mitochondrial Proteins

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

Suitable for use in Western Blot, Immunohistochemistry, ELISA
Dilution: ELISA: 1:1,000
Western Blot: 1:100-500
Immunohistochemistry: 1:50-100

48,455 Da[Similar Products]

glycine amidinotransferase (L-arginine:glycine amidinotransferase)

glycine amidinotransferase, mitochondrial; transamidinase; L-arginine:glycine amidinotransferase

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]

Function: 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. Ref.10 Ref.11 Ref.12

Catalytic activity: L-arginine + glycine = L-ornithine + guanidinoacetate. Ref.7

Pathway: Amine and polyamine biosynthesis; creatine biosynthesis; creatine from L-arginine and glycine: step 1/2.

Subunit structure: Homodimer. There is an equilibrium between the monomeric and dimeric forms, shifted towards the side of the monomer. Ref.7

Subcellular location: Isoform 1: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Note: Probably attached to the outer side of the inner membrane.Isoform 2: Cytoplasm.

Tissue specificity: Expressed in brain, heart, kidney, liver, lung, salivary gland and skeletal muscle tissue, with the highest expression in kidney. Biallelically expressed in placenta and fetal tissues. Ref.10 Ref.11 Ref.12

Induction: Expression is elevated in the myocardium during heart failure, and decreased in inter-uterine growth restriction (IUGR)-associated placenta. Ref.10 Ref.11

Domain: One chain folds into a compact single domain composed of repeating units, five beta-beta-alpha-beta modules, which surround the central active site.

Involvement in disease: Cerebral creatine deficiency syndrome 3 (CCDS3) [MIM:612718]: 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. Most patients develop a myopathy characterized by muscle weakness and atrophy later in life.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.14 Ref.15 Ref.19 Ref.20

Sequence similarities: Belongs to the amidinotransferase family.

Biophysicochemical propertiesKinetic parameters:KM=2.0 µM for arginine Ref.7 Ref.17KM=3.0 µM for glycineVmax=0.44 µmol/min/mg enzyme

Sequence caution: The sequence BAG60595.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

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