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Creatine is a derivative of the amino acid glycine.1 It is produced from glycine and L-arginine by arginine:glycine amidinotransferase (AGAT) and guanidinoacetate N-methyltransferase (GAMT) via a guanidinoacetate intermediate. Creatine circulates in the blood and enters tissues via the creatine transporter. In tissues, it is phosphorylated to creatine phosphate (Item Nos. 35598 | 37803), which is used as a reservoir of phosphoryl groups that can be transferred to ATP for the first few seconds of energy consumption, particularly in high-energy consumption tissues such as the brain and skeletal muscle.2 Decreased brain levels of creatine are associated with inborn errors of creatine metabolism characterized by deficiency of AGAT, GAMT, or the creatine transporter.1
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1. Arginine:Glycine amidinotransferase deficiency: The third inborn error of creatine metabolism in humans. Am. J. Hum. Genet. 69(5), 1127-1133 (2001).
2. Metabolism: Basic concepts and design. Biochemistry (2002).