Reaction: arginine + glycine => ornithine + guanidoacetate
- in pathway: Creatine metabolism
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).
Reaction - small molecule participants:
GAA [mitochondrial intermembrane space]
L-Orn [mitochondrial intermembrane space]
L-Arg [mitochondrial intermembrane space]
Gly [mitochondrial intermembrane space]
Reactome.org reaction link: R-HSA-71275
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Reaction input - small molecules:
L-argininium(1+)
glycine zwitterion
Reaction output - small molecules:
guanidinoacetic acid
L-ornithinium(1+)
Reactome.org link: R-HSA-71275