Glycocyamine is a metabolite of glycine and precursor in the biosynthesis of creatine.1,2 It is formed from glycine by glycine amidinotransferase (GATM) in the kidney and pancreas, transported to the liver, and methylated by guanidinoacetate N-methyltransferase (GAMT) to form creatine.1 Levels of glycocyamine are altered in individuals with cerebral creatine deficiency syndromes (CCDSs), inborn errors of metabolism characterized by deficiencies in GATM or GAMT.3
1.Ostojic, S.M.Cellular bioenergetics of guanidinoacetic acid: The role of mitochondriaJ. Bioenerg. Biomembr.47(5)369-372(2015) 2.Portocarero, N., and Braun, U.The physiological role of guanidinoacetic acid and its relationship with arginine in broiler chickensPoult. Sci.100(7)101203(2021) 3.Stockler, S., Schutz, P.W., and Salomons, G.S.Cerebral creatine deficiency syndromes: Clinical aspects, treatment and pathophysiologySubcell. Biochem.46149-166(2007)