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EC Number
Natural Substrates
Commentary (Nat. Sub.)
2.1.4.1
L-arginine + glycine
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2.1.4.1
L-arginine + glycine
first reaction in the de novo biosynthesis of creatine
2.1.4.1
L-arginine + glycine
nitrogen metabolism, putrescine biosynthesis
2.1.4.1
L-arginine + glycine
enzyme is involved in biosynthesis of creatine. Patients with AGAT deficiency show mental and motor retardation and severe delay in speech development. Both creatine and guanidinoacetate are decreased in body fluids of AGAT-deficient patients
2.1.4.1
L-ornithine + guanidinoacetate
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