• Target：
• GATM

• Fields：
• >>Glycine, serine and threonine metabolism;>>Arginine and proline metabolism;>>Metabolic pathways

• Gene Name：
• GATM AGAT

• Protein Name：
• GATM

• Human Gene Id：
• 2628

• Human Swiss Prot No：
• P50440

• Mouse Gene Id：
• 67092

• Mouse Swiss Prot No：
• Q9D964

• Rat Gene Id：
• 81660

• Rat Swiss Prot No：
• P50442

• Immunogen：
• Synthesized peptide derived from human GATM AA range: 223-273

• Specificity：
• This antibody detects endogenous levels of GATM at Human/Mouse/Rat

• Formulation：
• Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

• Source：
• Polyclonal, Rabbit,IgG

• Dilution：
• WB 1：500-2000

• Purification：
• The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

• Concentration：
• 1 mg/ml

• Storage Stability：
• -15°C to -25°C/1 year(Do not lower than -25°C)

• Molecular Weight(Da)：
• 47kD

• Background：
• 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：
• catalytic activity:L-arginine + glycine = L-ornithine + guanidinoacetate.,disease:Defects in GATM are the cause of L-arginine:glycine amidinotransferase deficiency (AGAT deficiency) [MIM:602360]. AGAT deficiency is a defect in creatine metabolism leading to mental retardation.,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.,pathway:Amine and polyamine biosynthesis; creatine biosynthesis; creatine from L-arginine and glycine: step 1/2.,similarity:Belongs to the amidinotransferase family.,subcellular location:The mitochondrial form is found in the intermembrane space probably attached to the outer side of the inner membrane.,subunit:Homodimer. There is an equilibrium between the monomeric and dimeric forms, shifted towards the side of the monomer.,tissue specificity:Kidney.,

• Subcellular Location：
• [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Probably attached to the outer side of the inner membrane.; [Isoform 2]: Cytoplasm.

• Expression：
• 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.

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