{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T12:59:49Z","timestamp":1762001989011,"version":"build-2065373602"},"reference-count":24,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,12,1]],"date-time":"2021-12-01T00:00:00Z","timestamp":1638316800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,8,30]],"date-time":"2021-08-30T00:00:00Z","timestamp":1630281600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100008731","name":"Nakatomi Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008731","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism Reports"],"published-print":{"date-parts":[[2021,12]]},"DOI":"10.1016\/j.ymgmr.2021.100799","type":"journal-article","created":{"date-parts":[[2021,9,3]],"date-time":"2021-09-03T20:27:39Z","timestamp":1630700859000},"page":"100799","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":0,"special_numbering":"C","title":["Influence of food on pharmacokinetics and pharmacodynamics of 4-phenylbutyrate in patients with urea cycle disorders"],"prefix":"10.1016","volume":"29","author":[{"given":"Yoko","family":"Nakajima","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shuhei","family":"Osaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadahaya","family":"Mizuno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katsuyuki","family":"Yokoi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Nakano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saeko","family":"Hirai","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuka","family":"Hiraoka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiki","family":"Miura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mitsuyoshi","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiroyuki","family":"Kusuhara","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hisamitsu","family":"Hayashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgmr.2021.100799_bb0005","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.ymgme.2014.08.001","article-title":"A longitudinal study of urea cycle disorders","volume":"113","author":"Batshaw","year":"2014","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0010","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1186\/2052-0492-2-22","article-title":"Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting","volume":"2","author":"Machado","year":"2014","journal-title":"J. Intens. Care"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0015","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1124\/dmd.32.1.10","article-title":"New secondary metabolites of phenylbutyrate in humans and rats","volume":"32","author":"Kasumov","year":"2004","journal-title":"Drug Metab. Dispos."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0020","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1203\/00006450-199102000-00009","article-title":"Phenylacetylglutamine may replace urea as a vehicle for waste nitrogen excretion","volume":"29","author":"Brusilow","year":"1991","journal-title":"Pediatr. Res."},{"year":"2009","series-title":"Labels for NDA 020572: BUPHENYL\u00ae (Sodium Phenylbutyrate)","key":"10.1016\/j.ymgmr.2021.100799_bb0025"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0030","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1016\/j.ymgme.2021.02.002","article-title":"A randomized trial to examine the impact of food on pharmacokinetics of 4-phenylbutyrate and change in amino acid availability after a single oral administration of sodium 4-phenylbutyrarte in healthy volunteers","volume":"132","author":"Osaka","year":"2021","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0035","doi-asserted-by":"crossref","first-page":"17075","DOI":"10.1038\/s41598-019-53628-x","article-title":"Effect of food on the pharmacokinetics and therapeutic efficacy of 4-phenylbutyrate in progressive familial intrahepatic cholestasis","volume":"9","author":"Nakano","year":"2019","journal-title":"Sci. Rep."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0040","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1055\/s-0031-1272831","article-title":"Progressive familial intrahepatic cholestasis (PFIC) type 1, 2, and 3: a review of the liver pathology findings","volume":"31","author":"Morotti","year":"2011","journal-title":"Semin. Liver Dis."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0045","series-title":"13. Familial Hepatocellular Cholestasis Liver Disease in Children","first-page":"199","author":"Suchy","year":"2014"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0050","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1186\/1750-1172-9-89","article-title":"Intractable itch relieved by 4-phenylbutyrate therapy in patients with progressive familial intrahepatic cholestasis type 1","volume":"9","author":"Hasegawa","year":"2014","journal-title":"Orphanet. J. Rare Dis."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0055","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1002\/hep.21630","article-title":"4-phenylbutyrate enhances the cell surface expression and the transport capacity of wild-type and mutated bile salt export pumps","volume":"45","author":"Hayashi","year":"2007","journal-title":"Hepatology"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0060","doi-asserted-by":"crossref","DOI":"10.1016\/j.jpeds.2013.12.032","article-title":"Improved liver function and relieved pruritus after 4-phenylbutyrate therapy in a patient with progressive familial intrahepatic cholestasis type 2","volume":"164","author":"Naoi","year":"2014","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0065","first-page":"43","article-title":"Barriers to drug adherence in the treatment of urea cycle disorders: assessment of patient, caregiver and provider perspectives","volume":"8","author":"Shchelochkov","year":"2016","journal-title":"Mol. Genet. Metab. Rep."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0070","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.ymgme.2015.11.005","article-title":"Glutamine and hyperammonemic crises in patients with urea cycle disorders","volume":"117","author":"Lee","year":"2016","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0075","first-page":"113","article-title":"Pairwise multiple comparison procedures with unequal N's and\/or variances: a Monte Carlo Study","volume":"1","author":"Games","year":"1976","journal-title":"J. Educ. Stat."},{"year":"2015","series-title":"Comparing Welch's ANOVA, a Kruskal-Wallis Test and Traditional ANOVA in Case of Heterogeneity of Variance","author":"Liu","key":"10.1016\/j.ymgmr.2021.100799_bb0080"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0085","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":"Haberle","year":"2019","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0090","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1186\/s12986-018-0271-1","article-title":"Branched-chain amino acids in health and disease: metabolism, alterations in blood plasma, and as supplements","volume":"15","author":"Holecek","year":"2018","journal-title":"Nutr. Metab. (Lond.)"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0095","doi-asserted-by":"crossref","first-page":"1547S","DOI":"10.1093\/jn\/135.6.1547S","article-title":"Hormonal and signaling role of branched-chain amino acids","volume":"135","author":"Nair","year":"2005","journal-title":"J. Nutr."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0100","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1301\/nr.2007.apr.167-172","article-title":"Lowered concentrations of branched-chain amino acids result in impaired growth and neurological problems: insights from a branched-chain alpha-keto acid dehydrogenase complex kinase-deficient mouse model","volume":"65","author":"Watford","year":"2007","journal-title":"Nutr. Rev."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0105","doi-asserted-by":"crossref","first-page":"912","DOI":"10.1016\/j.nucmedbio.2013.06.007","article-title":"Whole-body pharmacokinetics of HDAC inhibitor drugs, butyric acid, valproic acid and 4-phenylbutyric acid measured with carbon-11 labeled analogs by PET","volume":"40","author":"Kim","year":"2013","journal-title":"Nucl. Med. Biol."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0110","doi-asserted-by":"crossref","first-page":"2077","DOI":"10.1002\/hep.23589","article-title":"Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis","volume":"51","author":"McGuire","year":"2010","journal-title":"Hepatology"},{"key":"10.1016\/j.ymgmr.2021.100799_bb0115","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1016\/S0021-9258(18)70632-7","article-title":"Synthesis of phenylacetylglutamine by human tissue","volume":"229","author":"Moldave","year":"1957","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgmr.2021.100799_bb0120","series-title":"Protein and Amino Acid Requirements in Human Nutrition: Report of a Joint WHO\/FAO\/UNU Expert Consultation","first-page":"1","volume":"935","year":"2007"}],"container-title":["Molecular Genetics and Metabolism Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2214426921000938?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2214426921000938?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T12:52:38Z","timestamp":1762001558000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2214426921000938"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,12]]},"references-count":24,"alternative-id":["S2214426921000938"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgmr.2021.100799","relation":{},"ISSN":["2214-4269"],"issn-type":[{"type":"print","value":"2214-4269"}],"subject":[],"published":{"date-parts":[[2021,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Influence of food on pharmacokinetics and pharmacodynamics of 4-phenylbutyrate in patients with urea cycle disorders","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism Reports","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgmr.2021.100799","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"100799"}}