Arginine:Glycine amidinotransferase

L-Arginine:glycine amidinotransferase (AGAT; EC2.1.4.1) is the enzyme that catalyses the transfer of an amidino group from L-arginine to glycine. The products are L-ornithine and glycocyamine, also known as guanidinoacetate, the immediate precursor of creatine. Creatine and its phosphorylated form play a central role in the energy metabolism of muscle and nerve tissues. Creatine is in highest concentrations in the skeletal muscle, heart, spermatozoa and photoreceptor cells. Creatine helps buffer the rapid changes in ADP/ATP ratio in muscle and nerve cells during active periods. Creatine is also synthesized in other tissues, such as pancreas, kidneys, and liver, where amidinotransferase is located in the cytoplasm, including the intermembrane space of the mitochondria, of the cells that make up those tissues.