{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,7]],"date-time":"2026-10-07T21:34:31Z","timestamp":1791408871249,"version":"4.3.3"},"reference-count":234,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2024,4,1]],"date-time":"2024-04-01T00:00:00Z","timestamp":1711929600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2024,4,1]],"date-time":"2024-04-01T00:00:00Z","timestamp":1711929600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2024,1,25]],"date-time":"2024-01-25T00:00:00Z","timestamp":1706140800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001871","name":"Funda\u00e7\u00e3o para a Ci\u00eancia e a Tecnologia","doi-asserted-by":"publisher","award":["UIDB\/04138\/2020"],"award-info":[{"award-number":["UIDB\/04138\/2020"]}],"id":[{"id":"10.13039\/501100001871","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochemical Pharmacology"],"published-print":{"date-parts":[[2024,4]]},"DOI":"10.1016\/j.bcp.2024.116034","type":"journal-article","created":{"date-parts":[[2024,2,1]],"date-time":"2024-02-01T18:31:32Z","timestamp":1706812292000},"page":"116034","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":16,"special_numbering":"C","title":["Mitochondrial targets in hyperammonemia: Addressing urea cycle function to improve drug therapies"],"prefix":"10.1016","volume":"222","author":[{"given":"Marco F.","family":"Moedas","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo J.M.","family":"Sim\u00f5es","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margarida F.B.","family":"Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0005","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1016\/j.jhep.2010.11.031","article-title":"New insights on the mechanisms of valproate-induced hyperammonemia: inhibition of hepatic N-acetylglutamate synthase activity by valproyl-CoA","volume":"55","author":"Aires","year":"2011","journal-title":"J Hepatol"},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0010","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.2215\/CJN.10311013","article-title":"Urea and ammonia metabolism and the control of renal nitrogen excretion","volume":"10","author":"Weiner","year":"2015","journal-title":"Clin J Am Soc Nephrol."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0015","doi-asserted-by":"crossref","first-page":"1327","DOI":"10.1007\/s11011-015-9743-4","article-title":"Clinical science workshop: targeting the gut-liver-brain axis","volume":"31","author":"Patel","year":"2016","journal-title":"Metab Brain Dis."},{"key":"10.1016\/j.bcp.2024.116034_b0020","doi-asserted-by":"crossref","first-page":"193","DOI":"10.2147\/CEG.S160921","article-title":"A model of blood-ammonia homeostasis based on a quantitative analysis of nitrogen metabolism in the multiple organs involved in the production, catabolism, and excretion of ammonia in humans","volume":"11","author":"Levitt","year":"2018","journal-title":"Clin Exp Gastroenterol."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0025","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1111\/j.1478-3231.2010.02302.x","article-title":"Interorgan ammonia metabolism in liver failure: the basis of current and future therapies","volume":"31","author":"Wright","year":"2011","journal-title":"Liver Int."},{"key":"10.1016\/j.bcp.2024.116034_b0030","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1007\/s11011-014-9494-7","article-title":"Cerebral effects of ammonia in liver disease: current hypotheses","volume":"29","author":"Ott","year":"2014","journal-title":"Metab Brain Dis."},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0035","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/s41581-020-0267-8","article-title":"Consensus guidelines for management of hyperammonaemia in paediatric patients receiving continuous kidney replacement therapy","volume":"16","author":"Raina","year":"2020","journal-title":"Nat Rev Nephrol."},{"key":"10.1016\/j.bcp.2024.116034_b0040","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/bs.acc.2014.09.002","article-title":"Ammonia metabolism and hyperammonemic disorders","volume":"67","author":"Walker","year":"2014","journal-title":"Adv Clin Chem."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0045","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.abb.2013.04.009","article-title":"Clinical and biochemical aspects of primary and secondary hyperammonemic disorders","volume":"536","author":"H\u00e4berle","year":"2013","journal-title":"Arch Biochem Biophys."},{"key":"10.1016\/j.bcp.2024.116034_b0050","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1007\/s00467-011-1838-5","article-title":"Hyperammonemia in review: pathophysiology, diagnosis, and treatment","volume":"27","author":"Auron","year":"2012","journal-title":"Pediatr Nephrol."},{"key":"10.1016\/j.bcp.2024.116034_b0055","first-page":"S32","article-title":"P: 65 Drug-induced hyperammonaemia: data from vigibase, the WHO database","volume":"114","author":"Weiss","year":"2019","journal-title":"Am J Gastroenterol."},{"issue":"55","key":"10.1016\/j.bcp.2024.116034_b0060","first-page":"1","article-title":"Drug-associated hyperammonaemia: a Bayesian analysis of the WHO Pharmacovigilance Database","volume":"12","author":"Balcerac","year":"2022","journal-title":"Ann Intensive Care"},{"key":"10.1016\/j.bcp.2024.116034_b0065","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/S0009-8981(98)00019-9","article-title":"Solubility of NH3 and apparent pK of NH4+ in human plasma, isotonic salt solutions and water at 37\u00b0C","volume":"273","author":"Lang","year":"1998","journal-title":"Clin Chim Acta."},{"key":"10.1016\/j.bcp.2024.116034_b0070","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/j.addr.2015.04.009","article-title":"Recent advances in the treatment of hyperammonemia","volume":"90","author":"Matoori","year":"2015","journal-title":"Adv Drug Deliv Rev"},{"issue":"36","key":"10.1016\/j.bcp.2024.116034_b0075","doi-asserted-by":"crossref","first-page":"19184","DOI":"10.1074\/jbc.M116.740217","article-title":"Aquaporin 4 as a NH3 Channel","volume":"291","author":"Assentoft","year":"2016","journal-title":"J Biol Chem."},{"issue":"102027","key":"10.1016\/j.bcp.2024.116034_b0080","first-page":"1","article-title":"Aquaporins in insulin resistance and diabetes: More than channels!","volume":"44","author":"Galli","year":"2021","journal-title":"Redox Biol"},{"key":"10.1016\/j.bcp.2024.116034_b0085","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1007\/s10545-012-9546-2","article-title":"Ammonia toxicity to the brain","volume":"36","author":"Braissant","year":"2013","journal-title":"J Inherit Metab Dis."},{"key":"10.1016\/j.bcp.2024.116034_b0090","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.ab.2017.07.025","article-title":"Advances in methods for characterization of hepatic urea cycle enzymatic activity in HepaRG cells using UPLC-MS\/MS","volume":"535","author":"Moedas","year":"2017","journal-title":"Anal Biochem."},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0095","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1515\/CCLM.2002.112","article-title":"Mechanisms of hyperammonemia","volume":"40","author":"Bachmann","year":"2002","journal-title":"Clin Chem Lab Med."},{"key":"10.1016\/j.bcp.2024.116034_b0100","doi-asserted-by":"crossref","first-page":"S52","DOI":"10.1016\/j.ymgme.2003.10.014","article-title":"Ammonia toxicity to the brain and creatine","volume":"81","author":"Bachmann","year":"2004","journal-title":"Mol Gen Metab."},{"key":"10.1016\/j.bcp.2024.116034_b0105","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1016\/j.jceh.2014.12.003","article-title":"Gut microbiota: its role in hepatic encephalopathy","volume":"5","author":"Rai","year":"2015","journal-title":"J Clin Exp Hepatol."},{"key":"10.1016\/j.bcp.2024.116034_b0110","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1016\/j.copbio.2019.09.007","article-title":"Engineering commensal bacteria to rewire host\u2013microbiome interactions","volume":"62","author":"Hwang","year":"2020","journal-title":"Curr Opin Biotechnol."},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0115","doi-asserted-by":"crossref","first-page":"2841","DOI":"10.1172\/JCI79214","article-title":"Engineering the gut microbiota to treat hyperammonemia","volume":"125","author":"Shen","year":"2015","journal-title":"J Clin Invest."},{"issue":"12","key":"10.1016\/j.bcp.2024.116034_b0120","doi-asserted-by":"crossref","first-page":"2048","DOI":"10.1016\/j.cgh.2015.06.039","article-title":"Covert and Overt Hepatic Encephalopathy: Diagnosis and Management","volume":"13","author":"Patidar","year":"2015","journal-title":"Clin Gastroenterol Hepatol."},{"key":"10.1016\/j.bcp.2024.116034_b0125","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1016\/j.jhep.2022.03.014","article-title":"Heretical thoughts into hepatic encephalopathy","volume":"77","author":"Jalan","year":"2022","journal-title":"J Hepatol."},{"issue":"108","key":"10.1016\/j.bcp.2024.116034_b0130","first-page":"1","article-title":"Glutamine Synthetase: Localization Dictates Outcome","volume":"9","author":"Castegna","year":"2018","journal-title":"Genes"},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0135","doi-asserted-by":"crossref","first-page":"1063","DOI":"10.1515\/hsz-2021-0166","article-title":"Glutamine synthetase as a central element in hepatic glutamine and ammonia metabolism: novel aspects","volume":"402","author":"Frieg","year":"2021","journal-title":"Biol Chem"},{"key":"10.1016\/j.bcp.2024.116034_b0140","doi-asserted-by":"crossref","first-page":"1100","DOI":"10.1007\/s11064-022-03771-1","article-title":"Glial glutamine homeostasis in Health and Disease","volume":"48","author":"Andersen","year":"2023","journal-title":"Neurochem Res."},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0145","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1038\/s41556-018-0124-1","article-title":"The multifaceted contributions of mitochondria to cellular metabolism","volume":"20","author":"Spinelli","year":"2018","journal-title":"Nat Cell Biol."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0150","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1038\/ki.1987.44","article-title":"Pathways of urea transport in the mammalian kidney","volume":"31","author":"Knepper","year":"1987","journal-title":"Kidney Int."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0155","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1002\/j.2040-4603.2013.tb00491.x","article-title":"Renal ammonia metabolism and transport","volume":"3","author":"Weiner","year":"2013","journal-title":"Compr Physiol."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0160","doi-asserted-by":"crossref","first-page":"102","DOI":"10.2215\/CJN.04500422","article-title":"Acid-Base Disorders in the Critically Ill Patient","volume":"18","author":"Achanti","year":"2023","journal-title":"Clin J Am Soc Nephrol"},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0165","doi-asserted-by":"crossref","first-page":"E1127314","DOI":"10.1080\/23723556.2015.1127314","article-title":"A metabolic link between the urea cycle and cancer cell proliferation","volume":"3","author":"Nagamani","year":"2016","journal-title":"Mol Cell Oncol."},{"key":"10.1016\/j.bcp.2024.116034_b0170","doi-asserted-by":"crossref","DOI":"10.1016\/j.jhepr.2022.100479","article-title":"Lipid alterations in chronic liver disease and liver cancer","volume":"4","author":"Paul","year":"2022","journal-title":"JHEP Reports"},{"issue":"6365","key":"10.1016\/j.bcp.2024.116034_b0175","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1126\/science.aam9305","article-title":"Metabolic recycling of ammonia via glutamate dehydrogenase supports breast cancer biomass","volume":"358","author":"Spinelli","year":"2017","journal-title":"Science."},{"issue":"10","key":"10.1016\/j.bcp.2024.116034_b0180","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1038\/nrc.2016.71","article-title":"From Krebs to clinic: glutamine metabolism to cancer therapy","volume":"16","author":"Altman","year":"2016","journal-title":"Nat Rev Cancer."},{"key":"10.1016\/j.bcp.2024.116034_b0185","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.15252\/embj.201696151","article-title":"Cancer cell metabolism: the essential role of the nonessential amino acid, glutamine","volume":"36","author":"Zhang","year":"2017","journal-title":"EMBO J."},{"issue":"10","key":"10.1016\/j.bcp.2024.116034_b0190","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1038\/s41568-018-0054-z","article-title":"Rewiring urea cycle metabolism in cancer to support anabolism","volume":"18","author":"Keshet","year":"2018","journal-title":"Nat Rev Cancer."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0195","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1038\/s10038-019-0614-4","article-title":"Urea cycle disorders\u2014update","volume":"64","author":"Matsumoto","year":"2019","journal-title":"J Hum Genet."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0200","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.pcl.2017.11.004","article-title":"Inborn Errors of Metabolism with Hyperammonemia: Urea Cycle Defects and Related Disorders","volume":"65","author":"Summar","year":"2018","journal-title":"Pediatr Clin North Am."},{"issue":"32","key":"10.1016\/j.bcp.2024.116034_b0205","first-page":"1","article-title":"Suggested guidelines for the diagnosis and management of urea cycle disorders","volume":"7","author":"H\u00e4berle","year":"2012","journal-title":"Orphanet J Rare Dis"},{"key":"10.1016\/j.bcp.2024.116034_b0210","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1007\/8904_2017_13","article-title":"Normal neurological development during infancy despite massive hyperammonemia in early treated NAGS deficiency","volume":"37","author":"Reigstad","year":"2017","journal-title":"JIMD Reports."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0215","doi-asserted-by":"crossref","first-page":"1192","DOI":"10.1002\/jimd.12100","article-title":"Suggested guidelines for the diagnosis and management of urea cycle disorders: first revision","volume":"42","author":"H\u00e4berle","year":"2019","journal-title":"J Inherit Metab Dis."},{"key":"10.1016\/j.bcp.2024.116034_b0220","doi-asserted-by":"crossref","first-page":"87","DOI":"10.1146\/annurev.nutr.22.110801.140547","article-title":"Regulation of enzymes of the urea cycle and arginine metabolism","volume":"22","author":"Morris","year":"2002","journal-title":"Annu. Rev. Nutr."},{"key":"10.1016\/j.bcp.2024.116034_b0225","doi-asserted-by":"crossref","first-page":"S49","DOI":"10.1016\/j.ymgme.2010.02.020","article-title":"Creatine metabolism and the urea cycle","volume":"100","author":"Brosnan","year":"2010","journal-title":"Mol Gen Metab."},{"issue":"100791","key":"10.1016\/j.bcp.2024.116034_b0230","first-page":"1","article-title":"Creatine metabolism in patients with urea cycle disorders","volume":"29","author":"Ingoglia","year":"2021","journal-title":"Mol Genet Metab Reports"},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0235","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1007\/s00726-016-2183-6","article-title":"Creatine supplementation as a possible new therapeutic approach for fatty liver disease: early findings","volume":"48","author":"Deminice","year":"2016","journal-title":"Amino Acids."},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0240","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/nu15040863","article-title":"Creatine Supplementation to Improve Sarcopenia in Chronic Liver Disease: Facts and Perspectives","volume":"15","author":"Casciola","year":"2023","journal-title":"Nutrients."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0245","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/j.cmet.2015.05.023","article-title":"NAD+ metabolism and the control of energy homeostasis: a balancing act between mitochondria and the nucleus","volume":"22","author":"Cant\u00f3","year":"2015","journal-title":"Cell Metab."},{"issue":"51","key":"10.1016\/j.bcp.2024.116034_b0250","doi-asserted-by":"crossref","first-page":"42436","DOI":"10.1074\/jbc.R112.404863","article-title":"Mitochondrial protein acylation and intermediary metabolism: regulation by sirtuins and implications for metabolic disease","volume":"287","author":"Newman","year":"2012","journal-title":"J Biol Chem."},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0255","doi-asserted-by":"crossref","first-page":"270","DOI":"10.1038\/nrm3305","article-title":"A two-way street: reciprocal regulation of metabolism and signalling","volume":"13","author":"Wellen","year":"2012","journal-title":"Nat Rev Mol Cell Biol."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0260","doi-asserted-by":"crossref","first-page":"3169","DOI":"10.1002\/jcp.24054","article-title":"Metaboloepigenetics: interrelationships between energy metabolism and epigenetic control of gene expression","volume":"227","author":"Donohoe","year":"2012","journal-title":"J Cell Physiol."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0265","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1038\/nrendo.2015.181","article-title":"Protein acetylation in metabolism - metabolites and cofactors","volume":"12","author":"Menzies","year":"2015","journal-title":"Nat. Rev. Endocrinol."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0270","first-page":"E1","article-title":"Coenzyme A metabolism","volume":"248","author":"Robishaw","year":"1985","journal-title":"Am J Physiol Metab."},{"issue":"169","key":"10.1016\/j.bcp.2024.116034_b0275","first-page":"1","article-title":"The Regulatory Effects of Acetyl-CoA Distribution in the Healthy and Diseased Brain","volume":"12","author":"Ronowska","year":"2018","journal-title":"Front Cell Neurosci."},{"key":"10.1016\/j.bcp.2024.116034_b0280","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1016\/j.jhep.2018.06.023","article-title":"Urea cycle dysregulation in non-alcoholic fatty liver disease","volume":"69","author":"De Chiara","year":"2018","journal-title":"J Hepatol"},{"key":"10.1016\/j.bcp.2024.116034_b0285","article-title":"Role of ammonia in NAFLD: An unusual suspect","volume":"5","author":"Thomsen","year":"2023","journal-title":"J Hep Rep"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0290","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1016\/j.jhep.2016.07.046","article-title":"Cerebrospinal fluid metabolomics highlights dysregulation of energy metabolism in overt hepatic encephalopathy","volume":"65","author":"Weiss","year":"2016","journal-title":"J Hepatol."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0295","doi-asserted-by":"crossref","first-page":"1190","DOI":"10.1038\/s42255-020-00285-4","article-title":"Nuclear metabolism and the regulation of the epigenome","volume":"2","author":"Boon","year":"2020","journal-title":"Nat Metab"},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0300","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1038\/s41568-022-00543-5","article-title":"Acetyl-CoA metabolism in cancer","volume":"23","author":"Guertin","year":"2023","journal-title":"Nat Rev Cancer."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0305","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1016\/j.tibs.2006.11.006","article-title":"NAD+ metabolism in health and disease","volume":"32","author":"Belenky","year":"2007","journal-title":"Trends Biochem Sci."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0310","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1210\/er.2009-0026","article-title":"The secret life of NAD+: an old metabolite controlling new metabolic signaling pathways","volume":"31","author":"Houtkooper","year":"2010","journal-title":"Endocr Rev."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0315","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1089\/ars.2007.1672","article-title":"NAD+\/NADH and NADP+\/NADPH in cellular functions and cell death: regulation and biological consequences","volume":"10","author":"Ying","year":"2008","journal-title":"Antioxid Redox Signal."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0320","doi-asserted-by":"crossref","first-page":"2442","DOI":"10.1016\/j.bbadis.2015.08.017","article-title":"Sirtuin regulation in aging and injury","volume":"1852","author":"Poulose","year":"2015","journal-title":"Biochim Biophys Acta [BBA]-Molecular Basis Dis."},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0325","doi-asserted-by":"crossref","first-page":"464","DOI":"10.1016\/j.tcb.2014.04.002","article-title":"NAD+ and sirtuins in aging and disease","volume":"24","author":"Imai","year":"2014","journal-title":"Trends Cell Biol."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0330","doi-asserted-by":"crossref","first-page":"741","DOI":"10.1038\/nrc3340","article-title":"The NAD metabolome \u2212 a key determinant of cancer cell biology","volume":"12","author":"Chiarugi","year":"2012","journal-title":"Nat Rev Cancer."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0335","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1038\/nrd3793","article-title":"Kynurenines in the CNS: recent advances and new questions","volume":"12","author":"V\u00e9csei","year":"2013","journal-title":"Nat Rev Drug Discov."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0340","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1124\/jpet.107.120758","article-title":"NAD+ and vitamin B3: from metabolism to therapies","volume":"324","author":"Sauve","year":"2008","journal-title":"J Pharmacol Exp Ther."},{"issue":"142","key":"10.1016\/j.bcp.2024.116034_b0345","first-page":"1","article-title":"Transient decrease of hepatic NAD+ and amino acid alterations during treatment with valproate: new insights on drug-induced effects in vivo using targeted MS-based metabolomics","volume":"12","author":"Moedas","year":"2016","journal-title":"Metabolomics."},{"issue":"24","key":"10.1016\/j.bcp.2024.116034_b0350","doi-asserted-by":"crossref","first-page":"21767","DOI":"10.1074\/jbc.M110.213298","article-title":"Pathways and subcellular compartmentation of NAD biosynthesis in human cells: from entry of extracellular precursors to mitochondrial NAD generation","volume":"286","author":"Nikiforov","year":"2011","journal-title":"J Biol Chem."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0355","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1038\/nrendo.2015.117","article-title":"Physiological and pathophysiological roles of NAMPT and NAD metabolism","volume":"11","author":"Garten","year":"2015","journal-title":"Nat Rev Endocrinol."},{"issue":"D1","key":"10.1016\/j.bcp.2024.116034_b0360","doi-asserted-by":"crossref","first-page":"D1251","DOI":"10.1093\/nar\/gkv1003","article-title":"MitoCarta2. 0: an updated inventory of mammalian mitochondrial proteins","volume":"44","author":"Calvo","year":"2016","journal-title":"Nucleic Acids Res."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0365","doi-asserted-by":"crossref","first-page":"e113939","DOI":"10.1371\/journal.pone.0113939","article-title":"Metabolic profiling of alternative NAD biosynthetic routes in mouse tissues","volume":"9","author":"Mori","year":"2014","journal-title":"PLoS One."},{"issue":"1329","key":"10.1016\/j.bcp.2024.116034_b0370","first-page":"1","article-title":"Cellular and Mitochondrial NAD Homeostasis in Health and Disease","volume":"12","author":"Waddell","year":"2023","journal-title":"Cells"},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0375","doi-asserted-by":"crossref","DOI":"10.15252\/emmm.202113943","article-title":"NAD+ homeostasis in human health and disease","volume":"13","author":"Zapata-P\u00e9rez","year":"2021","journal-title":"EMBO Mol Med"},{"issue":"10","key":"10.1016\/j.bcp.2024.116034_b0380","doi-asserted-by":"crossref","first-page":"dmm047134","DOI":"10.1242\/dmm.047134","article-title":"Ammonia inhibits energy metabolism in astrocytes in a rapid and glutamate dehydrogenase 2-dependent manner","volume":"13","author":"Drews","year":"2020","journal-title":"Dis Model Mech"},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0385","first-page":"1","article-title":"The glutamate dehydrogenase pathway and its roles in cell and tissue biology in health and disease","volume":"6","author":"Plaitakis","year":"2017","journal-title":"Biology"},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0390","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1002\/jimd.12180","article-title":"Inborn errors of enzymes in glutamate metabolism","volume":"43","author":"Rumping","year":"2020","journal-title":"J Inherit Metab Dis."},{"issue":"10","key":"10.1016\/j.bcp.2024.116034_b0395","doi-asserted-by":"crossref","first-page":"1949","DOI":"10.2337\/db17-1561","article-title":"Liver glutamate dehydrogenase controls whole-body energy partitioning through amino acid\u2013derived gluconeogenesis and ammonia homeostasis","volume":"67","author":"Karaca","year":"2018","journal-title":"Diabetes."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0400","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.advenzreg.2009.10.029","article-title":"On the reversibility of glutamate dehydrogenase and the source of hyperammonemia in the hyperinsulinism\/hyperammonemia syndrome","volume":"50","author":"Treberg","year":"2009","journal-title":"Adv Enzyme Regul."},{"issue":"53","key":"10.1016\/j.bcp.2024.116034_b0405","first-page":"1","article-title":"Multiple forms of glutamate dehydrogenase in animals: structural determinants and physiological implications","volume":"5","author":"Bunik","year":"2016","journal-title":"Biology"},{"key":"10.1016\/j.bcp.2024.116034_b0410","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/j.abb.2017.10.010","article-title":"Regulation of glutamate dehydrogenase (GDH) in response to whole body freezing in wood frog liver linked to differential acetylation and ADP-ribosylation","volume":"636","author":"Green","year":"2017","journal-title":"Arch Biochem Biophys."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0415","first-page":"1","article-title":"Endogenous Activity of Glutamate Dehydrogenase from Different Tissues of Mice at Various Postnatal Ages: Effects of Substrates and Co-substrates on the Activity of the Enzyme from the Liver of Two Ages","volume":"33","author":"Wahlang","year":"2019","journal-title":"Indian J Gerontol"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0420","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1007\/s12640-011-9285-4","article-title":"The role of glutamate dehydrogenase in mammalian ammonia metabolism","volume":"21","author":"Spanaki","year":"2012","journal-title":"Neurotox Res."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0425","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1002\/glia.23105","article-title":"Expression of the human isoform of glutamate dehydrogenase, hGDH2, augments TCA cycle capacity and oxidative metabolism of glutamate during glucose deprivation in astrocytes","volume":"65","author":"Nissen","year":"2017","journal-title":"Glia."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0430","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1007\/s11064-017-2428-0","article-title":"Glutamate dehydrogenase, a complex enzyme at a crucial metabolic branch point","volume":"44","author":"Smith","year":"2019","journal-title":"Neurochem Res."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0435","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1007\/s11064-018-2575-y","article-title":"Localization of human glutamate dehydrogenases provides insights into their metabolic role and their involvement in disease processes","volume":"44","author":"Mathioudakis","year":"2019","journal-title":"Neurochem Res."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0440","doi-asserted-by":"crossref","first-page":"802","DOI":"10.1369\/0022155414549071","article-title":"Determination of glutamate dehydrogenase activity and its kinetics in mouse tissues using metabolic mapping (Quantitative Enzyme Histochemistry)","volume":"62","author":"Botman","year":"2014","journal-title":"J Histochem Cytochem."},{"issue":"474","key":"10.1016\/j.bcp.2024.116034_b0445","first-page":"1","article-title":"Sirt4: a multifaceted enzyme at the crossroads of mitochondrial metabolism and cancer","volume":"10","author":"Tomaselli","year":"2020","journal-title":"Front Oncol"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0450","doi-asserted-by":"crossref","first-page":"E1219","DOI":"10.1152\/ajpendo.00028.2010","article-title":"Systemic activation of glutamate dehydrogenase increases renal ammoniagenesis: implications for the hyperinsulinism\/hyperammonemia syndrome","volume":"298","author":"Treberg","year":"2010","journal-title":"Am J Physiol Endocrinol Metab."},{"issue":"248","key":"10.1016\/j.bcp.2024.116034_b0455","first-page":"1","article-title":"Characterizing the neurological phenotype of the hyperinsulinism hyperammonemia syndrome","volume":"17","author":"Rosenfeld","year":"2022","journal-title":"Orphanet J Rare Dis"},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0460","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1002\/jimd.12594","article-title":"Hyperinsulinism\/hyperammonemia syndrome caused by biallelic SLC25A36 mutation","volume":"46","author":"Safran","year":"2023","journal-title":"J Inherit Metab Dis."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0465","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.tcb.2020.09.010","article-title":"Metabolic Coordination of Cell Fate by \u03b1-Ketoglutarate-Dependent Dioxygenases","volume":"31","author":"Baksh","year":"2021","journal-title":"Trends Cell Biol."},{"key":"10.1016\/j.bcp.2024.116034_b0470","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/S0014-5793(03)01459-5","article-title":"Investigations by mass isotopomer analysis of the formation of D-2-hydroxyglutarate by cultured lymphoblasts from two patients with D-2-hydroxyglutaric aciduria","volume":"557","author":"Struys","year":"2004","journal-title":"FEBS Lett."},{"key":"10.1016\/j.bcp.2024.116034_b0475","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1038\/s42255-019-0032-0","article-title":"Metabolic signatures of cancer cells and stem cells","volume":"1","author":"Intlekofer","year":"2019","journal-title":"Nat Metab."},{"key":"10.1016\/j.bcp.2024.116034_b0480","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1038\/s41574-022-00773-5","article-title":"Metabolic determinants of tumour initiation","volume":"19","author":"Brunner","year":"2023","journal-title":"Nat Rev Endocrinol."},{"key":"10.1016\/j.bcp.2024.116034_b0485","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1038\/s41575-023-00754-7","article-title":"Nonalcoholic steatohepatitis-related hepatocellular carcinoma: pathogenesis and treatment","volume":"20","author":"Llovet","year":"2023","journal-title":"Nat Rev Gastroenterol Hepatol."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0490","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1016\/j.tcb.2020.02.005","article-title":"Nitrogen Metabolism in Cancer and Immunity","volume":"30","author":"Kurmi","year":"2020","journal-title":"Trends Cell Biol."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0495","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/j.gendis.2019.09.011","article-title":"Enzymatic and metabolic regulation of lysine succinylation","volume":"7","author":"Sreedhar","year":"2020","journal-title":"Genes Dis."},{"issue":"21","key":"10.1016\/j.bcp.2024.116034_b0500","doi-asserted-by":"crossref","first-page":"4063","DOI":"10.1007\/s00018-016-2280-4","article-title":"Post-translational modifications in mitochondria: protein signaling in the powerhouse","volume":"73","author":"Stram","year":"2016","journal-title":"Cell Mol Life Sci."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0505","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/j.cmet.2018.01.016","article-title":"The mitochondrial acylome emerges: proteomics, regulation by sirtuins, and metabolic and disease implications","volume":"27","author":"Carrico","year":"2018","journal-title":"Cell Metab."},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0510","first-page":"1645","article-title":"Mitochondrial sirtuins, Biochim Biophys Acta (BBA)-Proteins and Proteomics","volume":"1804","author":"Huang","year":"2010"},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0515","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/j.molcel.2011.01.002","article-title":"Sirt3 promotes the urea cycle and fatty acid oxidation during dietary restriction","volume":"41","author":"Hallows","year":"2011","journal-title":"Mol Cell."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0520","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.tibs.2010.09.003","article-title":"Regulation of intermediary metabolism by protein acetylation","volume":"36","author":"Guan","year":"2011","journal-title":"Trends Biochem Sci."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0525","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1016\/j.cell.2006.06.057","article-title":"SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells","volume":"126","author":"Haigis","year":"2006","journal-title":"Cell."},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0530","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1089\/ars.2017.7290","article-title":"The role of sirtuins in antioxidant and redox signaling","volume":"28","author":"Singh","year":"2018","journal-title":"Antioxid Redox Signal."},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0535","doi-asserted-by":"crossref","first-page":"1799","DOI":"10.1681\/ASN.2017111218","article-title":"Sirtuins in renal health and disease","volume":"29","author":"Morigi","year":"2018","journal-title":"J Am Soc Nephrol."},{"key":"10.1016\/j.bcp.2024.116034_b0540","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.ejmech.2018.10.028","article-title":"An overview of Sirtuins as potential therapeutic target: Structure, function and modulators","volume":"161","author":"Wang","year":"2019","journal-title":"Eur J Med Chem."},{"issue":"45","key":"10.1016\/j.bcp.2024.116034_b0545","first-page":"1","article-title":"Impact of sirtuin enzymes on the altered metabolic phenotype of malignantly transformed cells","volume":"10","author":"Ga\u00e1l","year":"2020","journal-title":"Front Oncol"},{"issue":"1004","key":"10.1016\/j.bcp.2024.116034_b0550","first-page":"1","article-title":"Impact of nutrition on short-term exercise-induced sirtuin regulation: Vegans differ from omnivores and lacto-ovo vegetarians","volume":"12","author":"Potthast","year":"2020","journal-title":"Nutrients"},{"issue":"20","key":"10.1016\/j.bcp.2024.116034_b0555","doi-asserted-by":"crossref","first-page":"13669","DOI":"10.1074\/jbc.M901921200","article-title":"Lysine 88 acetylation negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals","volume":"284","author":"Yu","year":"2009","journal-title":"J Biol Chem."},{"issue":"24156","key":"10.1016\/j.bcp.2024.116034_b0560","first-page":"1","article-title":"PGC-1\u03b1 Promotes Ureagenesis in Mouse Periportal Hepatocytes through SIRT3 and SIRT5 in Response to Glucagon","volume":"6","author":"Li","year":"2016","journal-title":"Sci Rep."},{"issue":"46","key":"10.1016\/j.bcp.2024.116034_b0565","doi-asserted-by":"crossref","first-page":"33583","DOI":"10.1074\/jbc.M705488200","article-title":"Regulation of insulin secretion by SIRT4, a mitochondrial ADP-ribosyltransferase","volume":"282","author":"Ahuja","year":"2007","journal-title":"J Biol Chem."},{"issue":"748","key":"10.1016\/j.bcp.2024.116034_b0570","first-page":"1","article-title":"Sirtuins and Insulin Resistance","volume":"9","author":"Zhou","year":"2018","journal-title":"Front Endocrinol"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0575","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1007\/s10545-017-0069-8","article-title":"Loss of sirtuin 4 leads to elevated glucose-and leucine-stimulated insulin levels and accelerated age-induced insulin resistance in multiple murine genetic backgrounds","volume":"41","author":"Huynh","year":"2018","journal-title":"J Inherit Metab Dis."},{"issue":"1006","key":"10.1016\/j.bcp.2024.116034_b0580","first-page":"1","article-title":"SIRT4 and its roles in energy and redox metabolism in health, disease and during exercise","volume":"10","author":"Han","year":"2019","journal-title":"Front Physiol"},{"issue":"783","key":"10.1016\/j.bcp.2024.116034_b0585","first-page":"1","article-title":"The roles of mitochondrial SIRT4 in cellular metabolism","volume":"9","author":"Min","year":"2019","journal-title":"Front Endocrinol"},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0590","doi-asserted-by":"crossref","first-page":"626","DOI":"10.1038\/s42255-023-00784-0","article-title":"Amino acids downregulate SIRT4 to detoxify ammonia through the urea cycle","volume":"5","author":"Hu","year":"2023","journal-title":"Nat Metab."},{"issue":"24","key":"10.1016\/j.bcp.2024.116034_b0595","doi-asserted-by":"crossref","first-page":"10239","DOI":"10.1074\/jbc.M117.785022","article-title":"Lysine desuccinylase SIRT5 binds to cardiolipin and regulates the electron transport chain","volume":"292","author":"Zhang","year":"2017","journal-title":"J Biol Chem."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0600","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1016\/j.cell.2009.02.026","article-title":"SIRT5 Deacetylates carbamoyl phosphate synthetase 1 and regulates the urea cycle","volume":"137","author":"Nakagawa","year":"2009","journal-title":"Cell."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0605","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/j.bbrc.2010.01.081","article-title":"Overexpression of SIRT5 confirms its involvement in deacetylation and activation of carbamoyl phosphate synthetase 1","volume":"393","author":"Ogura","year":"2010","journal-title":"Biochem Biophys Res Commun."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0610","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s13105-016-0492-6","article-title":"The role of sirtuins in cellular homeostasis","volume":"72","author":"Kupis","year":"2016","journal-title":"J Physiol Biochem."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0615","doi-asserted-by":"crossref","first-page":"578","DOI":"10.18632\/aging.100062","article-title":"Urea cycle regulation by mitochondrial sirtuin, SIRT5","volume":"1","author":"Nakagawa","year":"2009","journal-title":"Aging."},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0620","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1016\/j.cmet.2014.03.014","article-title":"Lysine glutarylation is a protein posttranslational modification regulated by SIRT5","volume":"19","author":"Tan","year":"2014","journal-title":"Cell Metab."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0625","doi-asserted-by":"crossref","first-page":"2308","DOI":"10.1074\/mcp.R114.046664","article-title":"Metabolic regulation by lysine malonylation, succinylation, and glutarylation","volume":"14","author":"Hirschey","year":"2015","journal-title":"Mol Cell Proteomics."},{"key":"10.1016\/j.bcp.2024.116034_b0630","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/bs.pmbts.2017.11.012","article-title":"Regulatory Effects of NAD+. Metabolic Pathways on Sirtuin Activity","volume":"154","author":"Zhang","year":"2018","journal-title":"Prog Mol Biol Transl Sci."},{"issue":"402","key":"10.1016\/j.bcp.2024.116034_b0635","first-page":"1","article-title":"The sirtuin family in health and disease","volume":"7","author":"Wu","year":"2022","journal-title":"Signal Transduct Target Ther."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0640","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1002\/jimd.12146","article-title":"Neuropsychological attributes of urea cycle disorders: A systematic review of the literature","volume":"42","author":"Waisbren","year":"2019","journal-title":"J Inherit Metab Dis."},{"issue":"R1","key":"10.1016\/j.bcp.2024.116034_b0645","doi-asserted-by":"crossref","first-page":"R42","DOI":"10.1093\/hmg\/ddz140","article-title":"Progress and challenges in development of new therapies for urea cycle disorders","volume":"28","author":"Soria","year":"2019","journal-title":"Hum Mol Genet."},{"issue":"1\u20132","key":"10.1016\/j.bcp.2024.116034_b0650","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.ymgme.2019.07.009","article-title":"Perspectives on urea cycle disorder management: results of a clinician survey","volume":"128","author":"Enns","year":"2019","journal-title":"Mol Genet Metab."},{"issue":"1\u20132","key":"10.1016\/j.bcp.2024.116034_b0655","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1016\/j.ymgme.2013.07.008","article-title":"The incidence of urea cycle disorders","volume":"110","author":"Summar","year":"2013","journal-title":"Mol. Genet. Metab."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0660","doi-asserted-by":"crossref","first-page":"1041","DOI":"10.1007\/s10545-015-9839-3","article-title":"The phenotypic spectrum of organic acidurias and urea cycle disorders. Part 1: the initial presentation","volume":"38","author":"K\u00f6lker","year":"2015","journal-title":"J Inherit Metab Dis"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0665","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1002\/ana.25492","article-title":"Impact of diagnosis and therapy on cognitive function in urea cycle disorders","volume":"86","author":"Posset","year":"2019","journal-title":"Ann Neurol."},{"issue":"187","key":"10.1016\/j.bcp.2024.116034_b0670","first-page":"1","article-title":"Valproic acid-induced hyperammonemic encephalopathy in a patient with bipolar disorder: a case report","volume":"10","author":"Wu","year":"2020","journal-title":"Brain Sci"},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b0675","doi-asserted-by":"crossref","first-page":"581","DOI":"10.1016\/S0006-291X(02)02696-7","article-title":"Cloning and expression of the human N-acetylglutamate synthase gene","volume":"299","author":"Caldovic","year":"2002","journal-title":"Biochem Biophys Res Commun."},{"issue":"100558","key":"10.1016\/j.bcp.2024.116034_b0680","first-page":"1","article-title":"Early care of N-acetyl glutamate synthase [NAGS] deficiency in three infants from an inbred family","volume":"22","author":"Peoc\u2019h","year":"2020","journal-title":"Mol Genet Metab Reports"},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0685","first-page":"1","article-title":"Management of late onset urea cycle disorders\u2014a remaining challenge for the intensivist?","volume":"11","author":"Redant","year":"2021","journal-title":"Ann Intensive Care"},{"key":"10.1016\/j.bcp.2024.116034_b0690","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.ymgme.2014.08.001","article-title":"and the Members of the Urea Cycle Disorders Consortium, A longitudinal study of urea cycle disorders","volume":"113","author":"Batshaw","year":"2014","journal-title":"Mol Genet Metab."},{"key":"10.1016\/j.bcp.2024.116034_b0695","doi-asserted-by":"crossref","first-page":"e597","DOI":"10.1016\/j.wneu.2020.09.027","article-title":"Incidence, Presentation, and Risk Factors for Sodium Valproate-Associated Hyperammonemia in Neurosurgical Patients: A Prospective","volume":"144","author":"Woo","year":"2020","journal-title":"Observational Study. World Neurosurg."},{"key":"10.1016\/j.bcp.2024.116034_b0700","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1136\/jcp-2022-208644","article-title":"Drug-induced hyperammonaemia","volume":"76","author":"Shakerdi","year":"2023","journal-title":"J Clin Pathol"},{"issue":"56","key":"10.1016\/j.bcp.2024.116034_b0705","article-title":"Capecitabine-induced hyperammonemic encephalopathy","volume":"114","author":"C. Cano","year":"2022","journal-title":"Rev Esp Enferm Dig."},{"issue":"19","key":"10.1016\/j.bcp.2024.116034_b0710","doi-asserted-by":"crossref","first-page":"3317","DOI":"10.1016\/0006-2952(86)90429-6","article-title":"Liver carbonic anhydrase and urea synthesis","volume":"35","author":"H\u00e4ussinger","year":"1986","journal-title":"The Effect of Diuretics, Biochem Pharmacol."},{"key":"10.1016\/j.bcp.2024.116034_b0715","unstructured":"LiverTox: Clinical and Research Information on Drug-Induced Liver Injury [Internet]. Bethesda (MD): National Institute of Diabetes and Digestive and Kidney Diseases; 2012-. Carbonic Anhydrase Inhibitors. [Updated 2021 Oct 13]. Available from: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK548664\/."},{"issue":"657","key":"10.1016\/j.bcp.2024.116034_b0720","doi-asserted-by":"crossref","first-page":"114873","DOI":"10.1016\/j.ab.2022.114873","article-title":"Blood ammonia in patients with chronic liver diseases: A better defined role in clinical practice","volume":"15","author":"Mallet","year":"2022","journal-title":"Anal Biochem"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0725","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1080\/10408347.2016.1153949","article-title":"The Measurement of Ammonia in Human Breath and its Potential in Clinical Diagnostics","volume":"46","author":"Brannelly","year":"2016","journal-title":"Crit Rev Anal Chem."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0730","doi-asserted-by":"crossref","first-page":"1268","DOI":"10.1177\/0300060517703278","article-title":"L-Carnitine supplementation to reverse hyperammonemia in a patient undergoing chronic valproic acid treatment: a case report","volume":"45","author":"Maldonado","year":"2017","journal-title":"J Int Med Res."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0735","doi-asserted-by":"crossref","first-page":"206","DOI":"10.1016\/j.ctrv.2007.11.003","article-title":"Valproic acid as epigenetic cancer drug: preclinical, clinical and transcriptional effects on solid tumors","volume":"34","author":"Duenas-Gonzalez","year":"2008","journal-title":"Cancer Treat Rev."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0740","doi-asserted-by":"crossref","first-page":"7667","DOI":"10.1007\/s11033-023-08568-1","article-title":"The evidence for repurposing anti-epileptic drugs to target cancer","volume":"50","author":"Aroosa","year":"2023","journal-title":"Mol Biol Rep"},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0745","doi-asserted-by":"crossref","first-page":"e006857","DOI":"10.1136\/jitc-2023-006857","article-title":"Valproic acid increases CAR T cell cytotoxicity against acute myeloid leukemia","volume":"11","author":"Wen","year":"2023","journal-title":"J Immunother Cancer."},{"key":"10.1016\/j.bcp.2024.116034_b0750","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.genhosppsych.2019.05.005","article-title":"Risk factors for valproic acid induced hyperammonemia and its association with cognitive functions","volume":"59","author":"Duman","year":"2019","journal-title":"Gen Hosp Psychiatry."},{"issue":"134","key":"10.1016\/j.bcp.2024.116034_b0755","first-page":"1","article-title":"Valproate-induced hyperammonemia-uncovering an underlying inherited metabolic disorder: a case report","volume":"12","author":"Mehta","year":"2018","journal-title":"J Med Case Rep."},{"key":"10.1016\/j.bcp.2024.116034_b0760","doi-asserted-by":"crossref","first-page":"e241429","DOI":"10.1136\/bcr-2020-241429","article-title":"Valproate-induced fatal acute hyperammonaemia-related encephalopathy in late-onset ornithine transcarbamylase deficiency","volume":"14","author":"Kazmierski","year":"2021","journal-title":"BMJ Case Rep CP."},{"key":"10.1016\/j.bcp.2024.116034_b0765","doi-asserted-by":"crossref","first-page":"e238023","DOI":"10.1136\/bcr-2020-238023","article-title":"Status epilepticus secondary to hyperammonaemia: a late presentation of an undiagnosed urea cycle defect","volume":"14","author":"Beddoes","year":"2021","journal-title":"BMJ Case Rep CP."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0770","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1111\/ane.12765","article-title":"Gait instability in valproate-treated patients: Call to measure ammonia levels","volume":"136","author":"Kipervasser","year":"2017","journal-title":"Acta Neurol Scand."},{"issue":"19","key":"10.1016\/j.bcp.2024.116034_b0775","first-page":"1","article-title":"Valproate-induced hyperammonemic encephalopathy: a case report","volume":"14","author":"Shah","year":"2020","journal-title":"J Med Case Rep."},{"key":"10.1016\/j.bcp.2024.116034_b0780","doi-asserted-by":"crossref","unstructured":"S. K\u00f6lker, J. H\u00e4berle, V. Walker, Urea Cycle Disorders. In Carla EM Hollak and Robin Lachmann (eds.). Inherited Metabolic Disease in Adults: A Clinical Guide. Oxford Univ Press (2016) 119-126.","DOI":"10.1093\/med\/9780199972135.003.0017"},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0785","doi-asserted-by":"crossref","first-page":"205","DOI":"10.1007\/s10545-008-0841-x","article-title":"Valproic acid metabolism and its effects on mitochondrial fatty acid oxidation: A review","volume":"31","author":"Silva","year":"2008","journal-title":"J Inherit Metab Dis."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0790","doi-asserted-by":"crossref","first-page":"792","DOI":"10.1016\/j.bcp.2009.10.011","article-title":"Inhibition of hepatic carnitine palmitoyl-transferase I [CPT IA] by valproyl-CoA as a possible mechanism of valproate-induced steatosis","volume":"79","author":"Aires","year":"2010","journal-title":"Biochem Pharmacol."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0795","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1111\/epi.12125","article-title":"Risk factors for hyperammonemia in pediatric patients with epilepsy","volume":"54","author":"Yamamoto","year":"2013","journal-title":"Epilepsia."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0800","doi-asserted-by":"crossref","first-page":"73","DOI":"10.9740\/mhc.2018.03.073","article-title":"Valproic acid\u2013induced hyperammonemia: Incidence, clinical significance, and treatment management","volume":"8","author":"Baddour","year":"2018","journal-title":"Ment Health Clin."},{"key":"10.1016\/j.bcp.2024.116034_b0805","doi-asserted-by":"crossref","DOI":"10.1016\/j.yebeh.2021.107967","article-title":"Valproic acid-induced encephalopathy: A review of clinical features, risk factors, diagnosis, and treatment","volume":"120","author":"Wu","year":"2021","journal-title":"Epilepsy Behav."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0810","doi-asserted-by":"crossref","first-page":"430","DOI":"10.7861\/clinmedicine.18-5-430","article-title":"Lesson of the month 1: Sodium valproate-induced encephalopathy","volume":"18","author":"Camilleri","year":"2018","journal-title":"Clin Med (Lond)"},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0815","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/MD.0000000000000066","article-title":"Risk factors of hyperammonemia in patients with epilepsy under valproic acid therapy","volume":"93","author":"Tseng","year":"2014","journal-title":"Medicine (Baltimore)"},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0820","doi-asserted-by":"crossref","first-page":"1891","DOI":"10.1176\/ajp.2006.163.11.1891","article-title":"An interaction between aspirin and valproate: the relevance of plasma protein displacement drug-drug interactions","volume":"163","author":"Sandson","year":"2006","journal-title":"Am J Psychiatry."},{"issue":"8","key":"10.1016\/j.bcp.2024.116034_b0825","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1212\/WNL.37.8.1392","article-title":"Clinical valproate toxicity induced by acetylsalicylic acid","volume":"37","author":"Goulden","year":"1987","journal-title":"Neurology"},{"key":"10.1016\/j.bcp.2024.116034_b0830","doi-asserted-by":"crossref","first-page":"1624","DOI":"10.1016\/j.cell.2013.11.037","article-title":"Declining NAD+ induces a pseudohypoxic state disrupting nuclear-mitochondrial communication during aging","volume":"155","author":"Gomes","year":"2013","journal-title":"Cell."},{"key":"10.1016\/j.bcp.2024.116034_b0835","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1038\/nrm.2016.23","article-title":"Mitonuclear communication in homeostasis and stress","volume":"17","author":"Quir\u00f3s","year":"2016","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"10.1016\/j.bcp.2024.116034_b0840","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1016\/j.bbalip.2007.01.010","article-title":"Studies on the extra-mitochondrial CoA-ester formation of valproic and \u03944-valproic acids","volume":"1771","author":"Aires","year":"2007","journal-title":"Biochim Biophys Acta - Mol Cell Biol Lipids."},{"issue":"Pt 3","key":"10.1016\/j.bcp.2024.116034_b0845","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1042\/bj3620755","article-title":"Complete beta-oxidation of valproate: cleavage of 3-oxovalproyl-CoA by a mitochondrial 3-oxoacyl-CoA thiolase","volume":"362","author":"Silva","year":"2002","journal-title":"Biochem J."},{"key":"10.1016\/j.bcp.2024.116034_b0850","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.taap.2015.08.022","article-title":"Mechanistic review of drug-induced steatohepatitis","volume":"289","author":"Schumacher","year":"2015","journal-title":"Toxicol. Appl. Pharmacol."},{"key":"10.1016\/j.bcp.2024.116034_b0855","doi-asserted-by":"crossref","first-page":"1740","DOI":"10.1016\/j.bcp.2011.07.103","article-title":"Valproic acid utilizes the isoleucine breakdown pathway for its complete \u03b2-oxidation","volume":"82","author":"Lu\u00eds","year":"2011","journal-title":"Biochem Pharmacol."},{"key":"10.1016\/j.bcp.2024.116034_b0860","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1124\/dmd.110.037606","article-title":"Role of isovaleryl-CoA dehydrogenase and short branched-chain acyl-CoA dehydrogenase in the metabolism of valproic acid: implications for the branched-chain amino acid oxidation pathway","volume":"39","author":"Lu\u00eds","year":"2011","journal-title":"Drug Metab Dispos."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0865","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1023\/A:1005398516208","article-title":"Valproate inhibits the mitochondrial pyruvate-driven oxidative phosphorylation in vitro","volume":"20","author":"Silva","year":"1997","journal-title":"J. Inherit. Metab. Dis."},{"issue":"23","key":"10.1016\/j.bcp.2024.116034_b0870","doi-asserted-by":"crossref","first-page":"7451","DOI":"10.1007\/s00018-021-03996-3","article-title":"Dihydrolipoamide dehydrogenase, pyruvate oxidation and acetylation-dependent mechanisms intersecting drug iatrogenesis","volume":"78","author":"Duarte","year":"2021","journal-title":"Cell Mol Life Sci"},{"key":"10.1016\/j.bcp.2024.116034_b0875","doi-asserted-by":"crossref","first-page":"1126","DOI":"10.1016\/j.bbabio.2007.06.007","article-title":"Valproic acid metabolites inhibit dihydrolipoyl dehydrogenase activity leading to impaired 2-oxoglutarate-driven oxidative phosphorylation","volume":"1767","author":"Lu\u00eds","year":"2007","journal-title":"Biochim Biophys Acta"},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b0880","doi-asserted-by":"crossref","first-page":"1912","DOI":"10.3390\/ijms18091912","article-title":"Mitochondrial liver toxicity of valproic acid and its acid derivatives is related to inhibition of \u03b1-lipoamide dehydrogenase","volume":"18","author":"Kudin","year":"2017","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.bcp.2024.116034_b0885","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1016\/j.neuroscience.2012.12.052","article-title":"The kynurenine pathway: a missing piece in the puzzle of valproate action?","volume":"234","author":"Maciejak","year":"2013","journal-title":"Neuroscience."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b0890","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1271\/bbb.120716","article-title":"Increased conversion of tryptophan to nicotinamide in rats by dietary valproate","volume":"77","author":"Shibata","year":"2013","journal-title":"Biosci Biotechnol Biochem."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b0895","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1006\/taap.1999.8683","article-title":"Peroxisome-proliferator regulates key enzymes of the tryptophan-NAD+ pathway","volume":"158","author":"Shin","year":"1999","journal-title":"Toxicol Appl Pharmacol."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0900","doi-asserted-by":"crossref","first-page":"207","DOI":"10.1016\/j.neulet.2005.09.030","article-title":"Functional characteristics of H+-dependent nicotinate transport in primary cultures of astrocytes from rat cerebral cortex","volume":"392","author":"Shimada","year":"2006","journal-title":"Neurosci Lett"},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0905","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1248\/bpb1978.13.301","article-title":"Carrier-mediated uptake of nicotinic acid by rat intestinal brush-border membrane vesicles and relation to monocarboxylic acid transport","volume":"13","author":"Simanjuntak","year":"1990","journal-title":"J Pharmacobiodyn."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0910","doi-asserted-by":"crossref","first-page":"C1773","DOI":"10.1152\/ajpcell.00409.2007","article-title":"Mechanism of nicotinic acid transport in human liver cells: experiments with HepG2 cells and primary hepatocytes","volume":"293","author":"Said","year":"2007","journal-title":"Am J Physiol Cell Physiol"},{"key":"10.1016\/j.bcp.2024.116034_b0915","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.eplepsyres.2015.07.002","article-title":"Pyruvate dehydrogenase complex deficiency and its relationship with epilepsy frequency\u2212An overview","volume":"116","author":"Bhandary","year":"2015","journal-title":"Epilepsy Res."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b0920","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1007\/s10545-015-9846-4","article-title":"Secondary NAD+ deficiency in the inherited defect of glutamine synthetase","volume":"38","author":"Hu","year":"2015","journal-title":"J Inherit Metab Dis."},{"issue":"24","key":"10.1016\/j.bcp.2024.116034_b0925","doi-asserted-by":"crossref","first-page":"6969","DOI":"10.1093\/emboj\/20.24.6969","article-title":"Valproic acid defines a novel class of HDAC inhibitors inducing differentiation of transformed cells","volume":"20","author":"G\u00f6ttlicher","year":"2001","journal-title":"EMBO J."},{"issue":"39","key":"10.1016\/j.bcp.2024.116034_b0930","doi-asserted-by":"crossref","first-page":"36734","DOI":"10.1074\/jbc.M101287200","article-title":"Histone deacetylase is a direct target of valproic acid, a potent anticonvulsant, mood stabilizer, and teratogen","volume":"276","author":"Phiel","year":"2001","journal-title":"J Biol Chem."},{"issue":"128","key":"10.1016\/j.bcp.2024.116034_b0935","first-page":"1","article-title":"Sodium valproate increases activity of the sirtuin pathway resulting in beneficial effects for spinocerebellar ataxia-3 in vivo","volume":"14","author":"Watchon","year":"2021","journal-title":"Mol Brain."},{"issue":"7","key":"10.1016\/j.bcp.2024.116034_b0940","first-page":"1","article-title":"L-carnitine does not improve valproic acid poisoning management: a cohort study with toxicokinetics and concentration\/effect relationships","volume":"12","author":"Nguyen","year":"2022","journal-title":"Ann Intensive Care"},{"key":"10.1016\/j.bcp.2024.116034_b0945","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1007\/s00213-022-06289-0","article-title":"Valproic acid\u2013induced hyperammonemia in neuropsychiatric disorders: a 2\u2013year clinical survey","volume":"240","author":"Hosseini","year":"2023","journal-title":"Psychopharmacology."},{"issue":"9300","key":"10.1016\/j.bcp.2024.116034_b0950","article-title":"An anticonvulsive drug, valproic acid (valproate), has effects on the biosynthesis of fatty acids and polyketides in microorganisms","volume":"10","author":"Poolchanuan","year":"2020","journal-title":"Sci Rep"},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b0955","doi-asserted-by":"crossref","first-page":"112","DOI":"10.32677\/IJCR.2020.v06.i03.005","article-title":"Valproate-induced hyperammonemic encephalopathy in a child without hepatic failure: A case report","volume":"6","author":"Dudipala","year":"2020","journal-title":"Indian J Case Reports"},{"key":"10.1016\/j.bcp.2024.116034_b0960","doi-asserted-by":"crossref","DOI":"10.1016\/j.bcp.2020.114278","article-title":"The therapeutic importance of acid-base balance","volume":"183","author":"Quade","year":"2021","journal-title":"Biochem Pharmacol."},{"key":"10.1016\/j.bcp.2024.116034_b0965","article-title":"Acute hyperammonemic encephalopathy after fluoropyrimidine-based chemotherapy A case series and review of the literature","volume":"96:22(e6874)","author":"Mitani","year":"2017","journal-title":"Medicine"},{"issue":"1641","key":"10.1016\/j.bcp.2024.116034_b0970","first-page":"1","article-title":"Fluoropyrimidine Modulation of the Anti-Tumor Immune Response - Prospects for Improved Colorectal Cancer Treatment","volume":"12","author":"Gmeiner","year":"2020","journal-title":"Cancers"},{"issue":"12","key":"10.1016\/j.bcp.2024.116034_b0975","doi-asserted-by":"crossref","first-page":"2505","DOI":"10.1038\/bjc.2013.262","article-title":"Pharmacogenetic variants in the DPYD, TYMS, CDA and MTHFR genes are clinically significant predictors of fluoropyrimidine toxicity","volume":"108","author":"Loganayagam","year":"2013","journal-title":"Br J Cancer."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0980","doi-asserted-by":"crossref","first-page":"414","DOI":"10.6004\/jadpro.2023.14.5.6","article-title":"Hyperammonemia Secondary to 5-Fluorouracil","volume":"14","author":"Scott","year":"2023","journal-title":"J Adv Pract Oncol."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b0985","doi-asserted-by":"crossref","first-page":"1996","DOI":"10.1002\/cam4.2036","article-title":"Hyperammonemia after capecitabine associated with occult impairment of the urea cycle","volume":"8","author":"Chu","year":"2019","journal-title":"Cancer Med."},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b0990","doi-asserted-by":"crossref","first-page":"3133","DOI":"10.1007\/s00277-023-05373-5","article-title":"Evaluating the efficacy and toxicity of dose adjusted pegylated L-asparaginase in combination with therapeutic drug monitoring","volume":"102","author":"Coe-Eisenberg","year":"2023","journal-title":"Ann Hematol"},{"key":"10.1016\/j.bcp.2024.116034_b0995","doi-asserted-by":"crossref","first-page":"1070069","DOI":"10.3389\/fonc.2023.1070069","article-title":"Possible mechanism of metabolic and drug resistance with L-asparaginase therapy in childhood leukaemia","volume":"13","author":"Zhou","year":"2023","journal-title":"Front Oncol"},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b1000","doi-asserted-by":"crossref","first-page":"107627","DOI":"10.1016\/j.ymgme.2023.107627","article-title":"Treatment and outcomes of symptomatic hyperammonemia following asparaginase therapy in children with acute lymphoblastic leukemia","volume":"139","author":"Lee","year":"2023","journal-title":"Mol Genet Metab"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b1005","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1177\/1078155214551590","article-title":"Hyperammonemia secondary to asparaginase: A case series","volume":"22","author":"Nussbaum","year":"2016","journal-title":"J Oncol Pharm Pract"},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b1010","doi-asserted-by":"crossref","DOI":"10.1002\/pbc.27239","article-title":"Pegylated asparaginase as cause of fatal hyperammonemia in patients with latent urea cycle disorder","volume":"65","author":"Peters","year":"2018","journal-title":"Pediatr Blood Cancer"},{"issue":"4","key":"10.1016\/j.bcp.2024.116034_b1015","doi-asserted-by":"crossref","first-page":"855","DOI":"10.1080\/10428194.2017.1352090","article-title":"Acute central nervous system complications and ammonium levels in adult patients with acute lymphoblastic leukemia receiving L-asparaginase","volume":"59","author":"Strickler","year":"2018","journal-title":"Leuk Lymphoma"},{"issue":"8473","key":"10.1016\/j.bcp.2024.116034_b1020","doi-asserted-by":"crossref","first-page":"162","DOI":"10.1016\/S0140-6736(86)92304-4","article-title":"Acute encephalopathy and hyperammonaemia complicating treatment of acute lymphoblastic leukaemia with asparaginase","volume":"327","author":"Leonard","year":"1986","journal-title":"Lancet."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b1025","doi-asserted-by":"crossref","first-page":"618","DOI":"10.1002\/jimd.12347","article-title":"Humanized liver mouse model with transplanted human hepatocytes from patients with ornithine transcarbamylase deficiency","volume":"44","author":"Sugahara","year":"2021","journal-title":"J Inherit Metab Dis."},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b1030","doi-asserted-by":"crossref","first-page":"646","DOI":"10.1002\/hep.32247","article-title":"Aquaporin 9 induction in human iPSC-derived hepatocytes facilitates modeling of ornithine transcarbamylase deficiency","volume":"76","author":"Laemmle","year":"2022","journal-title":"Hepatology."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b1035","first-page":"297","article-title":"Perspectives on the evaluation and adoption of complex in vitro models in drug development: Workshop with the FDA and the pharmaceutical industry (IQ MPS Affiliate)","volume":"39","author":"Baran","year":"2022","journal-title":"ALTEX - Alternatives to Animal Experimentation"},{"issue":"11","key":"10.1016\/j.bcp.2024.116034_b1040","first-page":"1","article-title":"Effects of a high protein diet and liver disease in an in silico model of human ammonia metabolism","volume":"16","author":"Griffin","year":"2019","journal-title":"Theor Biol Med Model"},{"key":"10.1016\/j.bcp.2024.116034_b1045","article-title":"Spatial multimodal analysis of transcriptomes and metabolomes in tissues","volume":"4","author":"Vicari","year":"2023","journal-title":"Nat Biotechnol"},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b1050","first-page":"317","article-title":"Clinical experience with glycerol phenylbutyrate in 20 patients with urea cycle disorders at a UK paediatric centre","volume":"64","author":"Yeo","year":"2023","journal-title":"JIMD Rep"},{"issue":"48","key":"10.1016\/j.bcp.2024.116034_b1055","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000031796","article-title":"A glance at transient hyperammonemia of the newborn: Pathophysiology, diagnosis, and treatment: A review","volume":"101","author":"Ni","year":"2022","journal-title":"Medicine (Baltimore)"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b1060","doi-asserted-by":"crossref","first-page":"100757","DOI":"10.1016\/j.aohep.2022.100757","article-title":"Current approaches to hepatic encephalopathy","volume":"27","author":"Vidal-Cevallos","year":"2022","journal-title":"Ann Hepatol"},{"key":"10.1016\/j.bcp.2024.116034_b1065","doi-asserted-by":"crossref","unstructured":"A.J.C. Kerbert, R. Jalan, Recent advances in understanding and managing hepatic encephalopathy in chronic liver disease, F1000Res. 9 (2020) F1000 Faculty Rev-312. https:\/\/doi.org\/10.12688\/f1000research.22183.1.","DOI":"10.12688\/f1000research.22183.1"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b1070","doi-asserted-by":"crossref","first-page":"1136","DOI":"10.1002\/jimd.12096","article-title":"Challenges in diagnosing and managing adult patients with urea cycle disorders","volume":"42","author":"Stepien","year":"2019","journal-title":"J Inherit Metab Dis."},{"issue":"2","key":"10.1016\/j.bcp.2024.116034_b1075","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1007\/s40268-021-00345-4","article-title":"Ammonia Removal by Metabolic Scavengers for the Prevention and Treatment of Hepatic Encephalopathy in Cirrhosis","volume":"21","author":"Butterworth","year":"2021","journal-title":"Drugs R d."},{"issue":"17","key":"10.1016\/j.bcp.2024.116034_b1080","doi-asserted-by":"crossref","first-page":"1660","DOI":"10.1056\/NEJMra1600561","article-title":"Hepatic encephalopathy","volume":"375","author":"Wijdicks","year":"2016","journal-title":"N Engl J Med"},{"issue":"43","key":"10.1016\/j.bcp.2024.116034_b1085","volume":"8","author":"H\u00e4ussinger","year":"2022","journal-title":"Hepatic Encephalopathy, Nat Rev Dis Primers"},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b1090","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/j.jhep.2022.06.001","article-title":"EASL Clinical Practice Guidelines on the management of hepatic encephalopathy. European Association for the Study of the Liver","volume":"77","year":"2022","journal-title":"J Hepatol"},{"issue":"140","key":"10.1016\/j.bcp.2024.116034_b1095","first-page":"1","article-title":"The pharmabiotic approach to treat hyperammonemia","volume":"10","author":"Liu","year":"2018","journal-title":"Nutrients."},{"issue":"100","key":"10.1016\/j.bcp.2024.116034_b1100","first-page":"1","article-title":"Formulation and clinical evaluation of sodium benzoate oral solution for the treatment of urea cycle disorders in pediatric patients","volume":"21","author":"Maines","year":"2020","journal-title":"AAPS PharmSciTech."},{"key":"10.1016\/j.bcp.2024.116034_b1105","doi-asserted-by":"crossref","first-page":"327","DOI":"10.2147\/TCRM.S12703","article-title":"Role of carglumic acid in the treatment of acute hyperammonemia due to N-acetylglutamate synthase deficiency","volume":"7","author":"H\u00e4berle","year":"2011","journal-title":"Ther Clin Risk Manag."},{"issue":"9","key":"10.1016\/j.bcp.2024.116034_b1110","first-page":"e3292","article-title":"Carglumic acid treatment of a patient with recurrent valproic acid-induced hyperammonemia: a rare case report","volume":"10","author":"Sattar","year":"2018","journal-title":"Cureus"},{"issue":"3","key":"10.1016\/j.bcp.2024.116034_b1115","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s40267-018-00595-4","article-title":"Carglumic acid in hyperammonaemia due to organic acidurias: a profile of its use in the EU","volume":"35","author":"Blair","year":"2019","journal-title":"Drugs Ther Perspect."},{"issue":"422","key":"10.1016\/j.bcp.2024.116034_b1120","first-page":"1","article-title":"Long-term effectiveness of carglumic acid in patients with propionic acidemia (PA) and methylmalonic acidemia (MMA): a randomized clinical trial","volume":"16","author":"Alfadhel","year":"2021","journal-title":"Orphanet J Rare Dis."},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b1125","first-page":"CD011451","article-title":"Acetyl-L-carnitine for patients with hepatic encephalopathy, Cochrane Database Syst","volume":"1","author":"Mart\u00ed-Carvajal","year":"2019","journal-title":"Rev"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b1130","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1007\/s11064-020-03044-9","article-title":"L-Aspartate, L-Ornithine and L-Ornithine-L-Aspartate [LOLA] and their impact on brain energy metabolism","volume":"45","author":"Das","year":"2020","journal-title":"Neurochem Res."},{"issue":"Suppl 1","key":"10.1016\/j.bcp.2024.116034_b1135","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1007\/s40265-018-1023-2","article-title":"Pharmacokinetic and pharmacodynamic properties of L-Ornithine-L-Aspartate (LOLA) in hepatic encephalopathy","volume":"79","author":"Kircheis","year":"2019","journal-title":"Drugs."},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b1140","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1111\/cts.13257","article-title":"Pharmacokinetics\/pharmacodynamics of L-ornithine phenylacetate in overt hepatic encephalopathy and the effect of plasma ammonia concentration reduction on clinical outcomes","volume":"15","author":"Safadi","year":"2022","journal-title":"Clin Transl Sci."},{"issue":"345","key":"10.1016\/j.bcp.2024.116034_b1145","first-page":"1","article-title":"Amino acid metabolism in health and disease","volume":"8","author":"Ling","year":"2023","journal-title":"Signal Transduct Target Ther"},{"issue":"6","key":"10.1016\/j.bcp.2024.116034_b1150","doi-asserted-by":"crossref","first-page":"1526","DOI":"10.1016\/j.jhep.2020.07.013","article-title":"Hepatic encephalopathy: novel insights into classification, pathophysiology and therapy","volume":"73","author":"Rose","year":"2020","journal-title":"J Hepatol."},{"issue":"5","key":"10.1016\/j.bcp.2024.116034_b1155","doi-asserted-by":"crossref","first-page":"1797","DOI":"10.1097\/HEP.0000000000000323","article-title":"AASLD Practice Guidance on the clinical assessment and management of nonalcoholic fatty liver disease","volume":"77","author":"Rinella","year":"2023","journal-title":"Hepatology"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b1160","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/j.jhep.2018.03.019","article-title":"European Association for the Study of the Liver, EASL- Clinical practice guidelines: management of hepatocellular carcinoma","volume":"69","year":"2018","journal-title":"J Hepatol"},{"issue":"1","key":"10.1016\/j.bcp.2024.116034_b1165","doi-asserted-by":"crossref","first-page":"283","DOI":"10.4143\/crt.2020.575","article-title":"Hyperammonemic encephalopathy mimicking ornithine transcarbamylase deficiency in fibrolamellar hepatocellular carcinoma: successful treatment with continuous venovenous hemofiltration and ammonia scavengers","volume":"53","author":"Lee","year":"2021","journal-title":"Cancer Res Treat."},{"issue":"18","key":"10.1016\/j.bcp.2024.116034_b1170","doi-asserted-by":"crossref","first-page":"3749","DOI":"10.1016\/j.molcel.2021.08.005","article-title":"The context-specific roles of urea cycle enzymes in tumorigenesis","volume":"81","author":"Hajaj","year":"2021","journal-title":"Mol Cell"}],"container-title":["Biochemical Pharmacology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006295224000170?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006295224000170?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T12:53:06Z","timestamp":1777380786000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0006295224000170"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,4]]},"references-count":234,"alternative-id":["S0006295224000170"],"URL":"https:\/\/doi.org\/10.1016\/j.bcp.2024.116034","relation":{},"ISSN":["0006-2952"],"issn-type":[{"value":"0006-2952","type":"print"}],"subject":[],"published":{"date-parts":[[2024,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Mitochondrial targets in hyperammonemia: Addressing urea cycle function to improve drug therapies","name":"articletitle","label":"Article Title"},{"value":"Biochemical Pharmacology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bcp.2024.116034","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2024 The Author(s). Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"116034"}}