Reactome | arginine + glycine => ornithine + guanidoacetate

arginine + glycine => ornithine + guanidoacetate

Stable Identifier

R-HSA-71275

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial intermembrane space

ReviewStatus

5/5

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arginine + glycine => ornithine + guanidoacetate

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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