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. Author manuscript; available in PMC: 2018 Jul 20.
Published in final edited form as: Expert Rev Endocrinol Metab. 2016 Sep 28;11(6):467–473. doi: 10.1080/17446651.2016.1239526

Figure 1.

Figure 1

The urea cycle and the mechanism of action of N-carbamoyl-L-glutamic acid (NCG). The enzymes in ovals and the solute transporters depicted by rectangles comprise the urea cycle. Thick gray line depicts mitochondrial membranes. NCG is a synthetic analogue of N-acetylglutamate and activates Carbamoyl phosphate synthase I (CPS1) and enhance ureagenesis through the urea cycle. AcCoA: Acetyl CoA; ASL: Argininosuccinate lyase; ARG: Arginase; ASS: Argininosuccinic acid synthetase; CPS I: Carbamoyl phosphate synthetase I; GPB: Glycerol phenylbutyrate; NAGS: N-acetylglutamate synthase; OTC: Ornithine transcarbamoylase; PAGN: Phenylacetylglutamine.