Answer
Phenylacetate, the active metabolite of glycerol phenylbutyrate, conjugates with glutamine via acetylation in the liver and kidneys to form PAGN, which is excreted by the kidneys and, like urea, carries two moles of nitrogen per mole, providing an alternative route for waste nitrogen excretion.
Sources for this answer
- RAVICTI 1.1 g/ml oral liquidImmedica · emc · SmPC §5.1 Pharmacodynamic properties · Version 7Document updated
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