Phenylbutyric acid (PBA), which is approved for treatment of urea cycle disorders (UCDs) as sodium phenylbutyrate (NaPBA), mediates waste nitrogen excretion via combination of PBA-derived phenylacetic acid with glutamine to form phenylactylglutamine (PAGN) that is excreted in urine. Glycerol phenylbutyrate (GPB), a liquid triglyceride pro-drug of PBA, containing no sodium and having favorable palatability, is being studied for treatment of hepatic encephalopathy (HE).
June 1, 2010
Author(s): Brendan McGuire, Igor Zupanets, Mark Lowe, Xunjun Xiao, Vasyliy Syplyviy, Jonathan Monteleone, Sharron Gargosky, Klara Dickinson, Antonia Martinez, Masoud Mokhtarani, Bruce Scharschmidt
Year: June 1, 2010