{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T23:22:15Z","timestamp":1781911335349,"version":"3.54.5"},"reference-count":31,"publisher":"Elsevier BV","issue":"1-2","license":[{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T00:00:00Z","timestamp":1689724800000},"content-version":"am","delay-in-days":321,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2022,9,1]],"date-time":"2022-09-01T00:00:00Z","timestamp":1661990400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"funder":[{"DOI":"10.13039\/100004319","name":"Pfizer","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004319","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R01 NS080866"],"award-info":[{"award-number":["R01 NS080866"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2022,9]]},"DOI":"10.1016\/j.ymgme.2022.07.001","type":"journal-article","created":{"date-parts":[[2022,7,8]],"date-time":"2022-07-08T08:49:59Z","timestamp":1657270199000},"page":"9-17","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["Biochemical and behavioural profile of NTBC treated Tyrosinemie type 1 mice"],"prefix":"10.1016","volume":"137","author":[{"given":"Willem G.","family":"van Ginkel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shelley R.","family":"Winn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Dudley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Destine","family":"Krenik","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruby","family":"Perez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Rimann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beat","family":"Th\u00f6ny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacob","family":"Raber","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cary O.","family":"Harding","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2022.07.001_bb0005","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1016\/0140-6736(92)92685-9","article-title":"Treatment of hereditary tyrosinaemia type I by inhibition of 4-hydroxyphenylpyruvate dioxygenase","volume":"340","author":"Lindstedt","year":"1992","journal-title":"Lancet"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0010","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.ymgme.2012.05.022","article-title":"Effect of nitisinone (NTBC) treatment on the clinical course of hepatorenal tyrosinemia in Quebec","volume":"107","author":"Larochelle","year":"2012","journal-title":"Mol.Genet.Metab."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0015","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1007\/s40272-019-00364-4","article-title":"Long-term outcomes and practical considerations in the pharmacological management of tyrosinemia type 1","volume":"21","author":"van Ginkel","year":"2019","journal-title":"Paediatr.Drugs"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0020","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/S2213-8587(21)00092-9","article-title":"Long-term safety and outcomes in hereditary tyrosinaemia type 1 with nitisinone treatment: a 15-year non-interventional, multicentre study","volume":"9","author":"Spiekerkoetter","year":"2021","journal-title":"Lancet Diabetes Endocrinol."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0025","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1007\/978-3-319-55780-9_10","article-title":"Neurological and neuropsychological problems in tyrosinemia type I patients","volume":"959","author":"van Ginkel","year":"2017","journal-title":"Adv.Exp.Med.Biol."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0030","doi-asserted-by":"crossref","DOI":"10.1186\/s13023-019-1259-2","article-title":"Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients","volume":"14","author":"van Vliet","year":"2019","journal-title":"Orphanet. J. Rare Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0035","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1007\/978-3-319-55780-9_20","article-title":"Fah knockout animals as models for therapeutic liver repopulation","volume":"959","author":"Grompe","year":"2017","journal-title":"Adv.Exp.Med.Biol."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0040","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1101\/gad.7.12a.2298","article-title":"Loss of fumarylacetoacetate hydrolase is responsible for the neonatal hepatic dysfunction phenotype of lethal albino mice","volume":"7","author":"Grompe","year":"1993","journal-title":"Genes Dev."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0045","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1006\/mgme.2001.3266","article-title":"Long-term therapy with NTBC and tyrosine-restricted diet in a murine model of hereditary tyrosinemia type I","volume":"75","author":"Al-Dhalimy","year":"2002","journal-title":"Mol.Genet.Metab."},{"issue":"5","key":"10.1016\/j.ymgme.2022.07.001_bb0050","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1007\/s10545-016-9949-6","article-title":"Tyrosinemia type I and not treatment with NTBC causes slower learning and altered behavior in mice","volume":"39","author":"Hillgartner","year":"2016","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0055","doi-asserted-by":"crossref","first-page":"2486","DOI":"10.3390\/nu11102486","article-title":"Blood and brain biochemistry and behaviour in NTBC and dietary treated tyrosinemia type 1 mice","volume":"11","author":"van Ginkel","year":"2019","journal-title":"Nutrients"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0060","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1038\/nn.3697","article-title":"Single app knock-in mouse models of Alzheimer\u2019s disease","volume":"17","author":"Saito","year":"2014","journal-title":"Nat.Neurosci."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0065","doi-asserted-by":"crossref","DOI":"10.1038\/s41598-021-83851-4","article-title":"Integrated analysis of behavioral, epigenetic, and gut microbiome analyses in App(NL-G-F), App(NL-F), and wild type mice","volume":"11","author":"Kundu","year":"2021","journal-title":"Sci.Rep."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0070","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1007\/s11011-017-0071-8","article-title":"Abnormal social behavior in mice with tyrosinemia type I is associated with an increase of myelin in the cerebral cortex","volume":"32","author":"Moore","year":"2017","journal-title":"Metab. Brain Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0075","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000017303","article-title":"Evolution of tyrosinemia type 1 disease in patients treated with nitisinone in Spain","volume":"98","author":"Couce","year":"2019","journal-title":"Medicine (Baltimore)"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0080","doi-asserted-by":"crossref","first-page":"3070","DOI":"10.3390\/nu13093070","article-title":"Physical growth of patients with hereditary tyrosinaemia type I: a single-centre retrospective study","volume":"13","author":"Yilmaz","year":"2021","journal-title":"Nutrients"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0085","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1046\/j.0306-5251.2001.01421.x","article-title":"Pharmacokinetics and pharmacodynamics of NTBC (2-(2-nitro-4-fluoromethylbenzoyl)-1,3-cyclohexanedione) and mesotrione, inhibitors of 4-hydroxyphenyl pyruvate dioxygenase (HPPD) following a single dose to healthy male volunteers","volume":"52","author":"Hall","year":"2001","journal-title":"Br.J.Clin.Pharmacol."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0090","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1111\/j.1365-277X.2011.01215.x","article-title":"Diurnal variation of phenylalanine concentrations in tyrosinaemia type 1: should we be concerned?","volume":"25","author":"Daly","year":"2012","journal-title":"J.Hum.Nutr.Diet."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0095","first-page":"117","article-title":"Infants with Tyrosinemia type 1: should phenylalanine be supplemented?","volume":"18","author":"van Vliet","year":"2014","journal-title":"JIMD Rep."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0100","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1007\/s10545-013-9675-2","article-title":"Pharmacologic inhibition of L-tyrosine degradation ameliorates cerebral dopamine deficiency in murine phenylketonuria (PKU)","volume":"37","author":"Harding","year":"2014","journal-title":"J.Inherit.Metab.Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0105","first-page":"49","article-title":"What is the best blood sampling time for metabolic control of phenylalanine and tyrosine concentrations in tyrosinemia type 1 patients?","volume":"36","author":"van Dam","year":"2017","journal-title":"JIMD Rep."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0110","doi-asserted-by":"crossref","first-page":"202","DOI":"10.1007\/s11064-013-1207-9","article-title":"L-tyrosine induces DNA damage in brain and blood of rats","volume":"39","author":"De Pra","year":"2014","journal-title":"Neurochem.Res."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0115","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1186\/1750-1172-9-73","article-title":"Brain-blood amino acid correlates following protein restriction in murine maple syrup urine disease","volume":"9","author":"Vogel","year":"2014","journal-title":"Orphanet. J.Rare Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0120","doi-asserted-by":"crossref","first-page":"1292","DOI":"10.3945\/ajcn.116.135996","article-title":"Therapeutic brain modulation with targeted large neutral amino acid supplements in the Pah-enu2 phenylketonuria mouse model","volume":"104","author":"van Vliet","year":"2016","journal-title":"Am.J.Clin.Nutr."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0125","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1186\/1750-1172-8-133","article-title":"Phenylketonuria: reduced tyrosine brain influx relates to reduced cerebral protein synthesis","volume":"8","author":"de Groot","year":"2013","journal-title":"Orphanet. J.Rare Dis."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0130","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/j.ymgme.2010.11.003","article-title":"Increase of CSF tyrosine and impaired serotonin turnover in tyrosinemia type I","volume":"102","author":"Thimm","year":"2011","journal-title":"Mol.Genet.Metab."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0135","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1007\/s00702-014-1238-7","article-title":"Complex molecular regulation of tyrosine hydroxylase","volume":"121","author":"Tekin","year":"2014","journal-title":"J. Neural. Transm.(Vienna)"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0140","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1002\/ajmg.b.32764","article-title":"Tyrosinemia type 1 and symptoms of ADHD: biochemical mechanisms and implications for treatment and prognosis","volume":"183","author":"Barone","year":"2020","journal-title":"Am.J.Med.Genet.B.Neuropsychiatr.Genet."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0145","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1007\/s002130050327","article-title":"Behavioral phenotypes of inbred mouse strains: implications and recommendations for molecular studies","volume":"132","author":"Crawley","year":"1997","journal-title":"Psychopharmacology"},{"key":"10.1016\/j.ymgme.2022.07.001_bb0150","doi-asserted-by":"crossref","first-page":"593","DOI":"10.1042\/BJ20042023","article-title":"Mouse behavioural analysis in systems biology","volume":"389","author":"Van Meer","year":"2005","journal-title":"Biochem.J."},{"key":"10.1016\/j.ymgme.2022.07.001_bb0155","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1111\/gbb.12364","article-title":"Standardized experiments in mutant mice reveal behavioural similarity on 129S5 and C57BL\/6J backgrounds","volume":"16","author":"van de Lagemaat","year":"2017","journal-title":"Genes Brain Behav."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719222003626?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719222003626?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T23:52:17Z","timestamp":1761090737000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719222003626"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,9]]},"references-count":31,"journal-issue":{"issue":"1-2","published-print":{"date-parts":[[2022,9]]}},"alternative-id":["S1096719222003626"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2022.07.001","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2022,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Biochemical and behavioural profile of NTBC treated Tyrosinemie type 1 mice","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2022.07.001","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 Published by Elsevier Inc.","name":"copyright","label":"Copyright"}]}}