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[DOI] [PMC free article] [PubMed] [Google Scholar] 104.Cazzola M., Calzetta L., Page C., Jardim J., Chuchalin A.G., Rogliani P., Gabriella Matera M

This study found that in animals treated with 2-Aminobicyclo [2, 2, 1] heptane-2-carboxylic acid (BCH), a nonmetabolic analogue of leucine, the renal arteriovenous ammonia level change was greater than in the liver, indicating that hyperammonemia in GDH-HI patients is mainly induced due to increased renal ammonia production, instead of abnormalities in urea synthesis in the liver