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