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Glycerol phenylbutyrate for the chronic management of urea cycle disorders

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Elżbieta Supruniuk, Ewa Żebrowska & Adrian Chabowski. (2023) Branched chain amino acids—friend or foe in the control of energy substrate turnover and insulin sensitivity?. Critical Reviews in Food Science and Nutrition 63:15, pages 2559-2597.
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Luis Peña-Quintana, Marta Llarena, Desiderio Reyes-Suárez & Luis Aldámiz-Echevarria. (2017) Profile of sodium phenylbutyrate granules for the treatment of urea-cycle disorders: patient perspectives. Patient Preference and Adherence 11, pages 1489-1496.
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Javier De Las Heras, Luis Aldámiz-Echevarría, María-Luz Martínez-Chantar & Teresa C. Delgado. (2017) An update on the use of benzoate, phenylacetate and phenylbutyrate ammonia scavengers for interrogating and modifying liver nitrogen metabolism and its implications in urea cycle disorders and liver disease. Expert Opinion on Drug Metabolism & Toxicology 13:4, pages 439-448.
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Cristel C. Chapel-Crespo, George A. Diaz & Kimihiko Oishi. (2016) Efficacy of N-carbamoyl-L-glutamic acid for the treatment of inherited metabolic disorders. Expert Review of Endocrinology & Metabolism 11:6, pages 467-473.
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Articles from other publishers (1)

Fernando Andrade, Isidro Vitoria, Elena Martín Hernández, Guillem Pintos-Morell, Patricia Correcher, Rocío Puig-Piña, Pilar Quijada-Fraile, Luis Peña-Quintana, Ana Mª Marquez, Olatz Villate, Mª Teresa García Silva, Javier de las Heras, Leticia Ceberio, Esmeralda Rodrigues, Teresa Almeida Campos, Raquel Yahyaoui, Javier Blasco, Inmaculada Vives-Piñera, David Gil, Mireia del Toro, Mónica Ruiz-Pons, Elvira Cañedo, Miguel Angel Barba Romero, Mª Concepción García-Jiménez & Luis Aldámiz-Echevarría. (2019) Quantification of urinary derivatives of Phenylbutyric and Benzoic acids by LC-MS/MS as treatment compliance biomarkers in Urea Cycle disorders. Journal of Pharmaceutical and Biomedical Analysis 176, pages 112798.
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