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. Author manuscript; available in PMC: 2018 Aug 25.
Published in final edited form as: Genet Med. 2017 Oct 12;20(7):708–716. doi: 10.1038/gim.2017.167

Figure 1. Alternative pathways for nitrogen disposal.

Figure 1

With a block in ureagenesis, excretion of conjugates of amino acids can serve as an alternative mechanism for disposal of nitrogen. Benzoate and phenylacetate can be conjugated with glycine and glutamine, respectively to generate hippuric acid and phenylacetylglutamine. The nitrogen atoms scavenged by these medications is depicted by grey circles. Whereas one molecule of benzoate scavenges one atom of nitrogen, one molecule of phenylacetate scavenges two atoms of nitrogen.