| 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. |
| Function | Transamidinase that catalyzes the transfer of the amidino group of L-arginine onto the amino moiety of acceptor metabolites such as glycine, beta-alanine, gamma-aminobutyric acid (GABA) and taurine yielding the corresponding guanidine derivatives. Catalyzes the rate-limiting step of creatine biosynthesis, namely the transfer of the amidino group from L-arginine to glycine to generate guanidinoacetate, which is then methylated by GAMT to form creatine. Provides creatine as a source for ATP generation in tissues with high energy demands, in particular skeletal muscle, heart and brain (Probable). |
| Protein Name | Glycine Amidinotransferase - MitochondrialL-Arginine -Glycine AmidinotransferaseTransamidinase |
| Database Links | Reactome: R-HSA-71288 |
| Cellular Localisation | Isoform 1: Mitochondrion Inner MembranePeripheral Membrane ProteinIntermembrane SideProbably Attached To The Outer Side Of The Inner MembraneIsoform 2: Cytoplasm |
| Alternative Protein Names | Glycine Amidinotransferase - Mitochondrial proteinL-Arginine -Glycine Amidinotransferase proteinTransamidinase proteinGATM proteinAGAT protein |
Information sourced from Uniprot.org
