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).
Humm, A, Fritsche, E, Steinbacher, S, Huber, R
Humm, A, Fritsche, E, Mann, K, Gohl, M, Huber, R
Item, CB, Stockler-Ipsiroglu, S, Stromberger, C, Muhl, A, Alessandri, MG, Bianchi, MC, Tosetti, M, Fornai, F, Cioni, G
glycine amidinotransferase activity of GATM dimer [mitochondrial intermembrane space]
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