arginine + glycine => ornithine + guanidoacetate

Stable Identifier
Reaction [transition]
Homo sapiens
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Glycine amindinotransferase, localized to the mitochondrial intermembrane space, catalyzes the reaction of arginine and glycine to form guanidinoacetate and ornithine. The active form of the enzyme is a dimer (Humm et al. 1997 {EMBO J]; Humm et al 1997 [Biochem J]). Its function in vivo has been confirmed by molecular and biochemical studies of patients deficient in the enzyme (Item et al. 2001).
Literature References
PubMed ID Title Journal Year
9148748 Recombinant expression and isolation of human L-arginine:glycine amidinotransferase and identification of its active-site cysteine residue

Huber, R, Humm, A, Fritsche, E, Gohl, M, Mann, K

Biochem J 1997
9218780 Crystal structure and mechanism of human L-arginine:glycine amidinotransferase: a mitochondrial enzyme involved in creatine biosynthesis.

Steinbacher, S, Huber, R, Humm, A, Fritsche, E

EMBO J 1997
11555793 Arginine:glycine amidinotransferase deficiency: the third inborn error of creatine metabolism in humans

Cioni, G, Stockler-Ipsiroglu, S, Stromberger, C, Bianchi, MC, Item, CB, Tosetti, M, Alessandri, MG, Muhl, A, Fornai, F

Am J Hum Genet 2001
Catalyst Activity

glycine amidinotransferase activity of GATM dimer [mitochondrial intermembrane space]

Orthologous Events
Cross References
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