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N-Acetylglutamate synthase deficiency
Carbamyl Phosphate Synthase 1 is an enzyme found in mitochondrial matrix and it catalyzes the very first reaction of the Urea cycle, in which Carbamyl Phosphate is produced. Carbamyl Phosphate Synthase 1, abbreviated as CPS1, is activated by its biologically natural activator N-Acetyl Glutamate, which in turn is synthesized from Acetyl-CoA and Glutamic Acid in the reaction catalyzed by N-Acetyl Glutamate Synthase, commonly called NAGS.
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