|Application ||WB, E|
|Other Accession||P50442, P50441, Q9D964, Q4R806, Q2HJ74, NP_001473.1|
|Predicted||Bovine, Monkey, Mouse, Pig, Rat|
|Calculated MW||48455 Da|
|Antigen Region||350-378 aa|
|Other Names||Glycine amidinotransferase, mitochondrial, L-arginine:glycine amidinotransferase, Transamidinase, GATM, AGAT|
|Target/Specificity||This GATM antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 350-378 amino acids from the C-terminal region of human GATM.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||GATM Antibody(C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Isoform 1: Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side. Note=Probably attached to the outer side of the inner membrane|
|Tissue Location||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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Provided below are standard protocols that you may find useful for product applications.
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.
Kottgen, A., et al. Nat. Genet. 42(5):376-384(2010)
Kottgen, A., et al. Nat. Genet. 41(6):712-717(2009)
Lion-Francois, L., et al. Neurology 67(9):1713-1714(2006)
Cullen, M.E., et al. Circulation 114 (1 SUPPL), I16-I20 (2006) :
Battini, R., et al. J. Pediatr. 148(6):828-830(2006)
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