{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,27]],"date-time":"2026-02-27T18:17:23Z","timestamp":1772216243185,"version":"3.50.1"},"reference-count":23,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2004,4,1]],"date-time":"2004-04-01T00:00:00Z","timestamp":1080777600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2004,4]]},"DOI":"10.1016\/j.ymgme.2003.11.017","type":"journal-article","created":{"date-parts":[[2004,2,17]],"date-time":"2004-02-17T10:08:37Z","timestamp":1077012517000},"page":"79-85","source":"Crossref","is-referenced-by-count":87,"special_numbering":"S","title":["Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients"],"prefix":"10.1016","volume":"81","author":[{"given":"Fernando","family":"Scaglia","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Susan","family":"Carter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William E","family":"O\u2019Brien","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brendan","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2003.11.017_BIB1","series-title":"The Molecular and Metabolic Basis of Inherited Disease","first-page":"1187","article-title":"Urea cycle enzymes","author":"Brusilow","year":"1995"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB2","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."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB3","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1016\/S0022-3476(05)81200-4","article-title":"Plasma glutamine concentration: a guide in the management of urea cycle disorders","volume":"121","author":"Maestri","year":"1992","journal-title":"J. Pediatr."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB4","doi-asserted-by":"crossref","first-page":"8021","DOI":"10.1073\/pnas.140082197","article-title":"In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle","volume":"97","author":"Lee","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB5","first-page":"E801","article-title":"Phenylbutyrate-induced glutamine depletion in humans: effect on leucine metabolism","volume":"274","author":"Darmaun","year":"1998","journal-title":"Am. J. Physiol."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB6","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/0016-5085(80)90667-8","article-title":"Effect of spontaneous portal-systemic shunting on plasma insulin and amino acid concentrations","volume":"78","author":"Iwasaki","year":"1980","journal-title":"Gastroenterology"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB7","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1620\/tjem.158.171","article-title":"Plasma amino acid abnormalities in liver disease: comparative analysis of idiopathic portal hypertension, extrahepatic portal occlusion and liver cirrhosis","volume":"158","author":"Ouchi","year":"1989","journal-title":"Tohoku J. Exp. Med."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB8","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1111\/j.1753-4887.1990.tb02967.x","article-title":"Is glutamine a conditionally essential amino acid?","volume":"48","author":"Lacey","year":"1990","journal-title":"Nutr. Rev."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB9","doi-asserted-by":"crossref","first-page":"628","DOI":"10.1053\/meta.2002.32018","article-title":"Does enteral glutamine modulate whole-body leucine kinetics in hypercatabolic dogs in a fed state?","volume":"51","author":"Humbert","year":"2002","journal-title":"Metabolism"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB10","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1096\/fasebj.10.8.8666159","article-title":"Glutamine nutrition and metabolism: where do we go from here?","volume":"10","author":"Neu","year":"1996","journal-title":"FASEB J."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB11","first-page":"E663","article-title":"Response of glutamine metabolism to exogenous glutamine in humans","volume":"269","author":"Hankard","year":"1995","journal-title":"Am. J. Physiol."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB12","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1016\/0140-6736(90)90811-I","article-title":"Alpha-ketoglutarate and postoperative muscle catabolism","volume":"335","author":"Wernerman","year":"1990","journal-title":"Lancet"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB13","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/S0140-6736(89)91254-3","article-title":"Effect of parenteral glutamine peptide supplements on muscle glutamine loss and nitrogen balance after major surgery","volume":"1","author":"Stehle","year":"1989","journal-title":"Lancet"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB14","series-title":"International Symposium on Metabolism and Clinical Implications of Branched-Chain Amino and Keto-Acids","first-page":"205","article-title":"Regulatory effects of leucine on carbohydrate and protein metabolism","author":"Goldberg","year":"1981"},{"key":"10.1016\/j.ymgme.2003.11.017_BIB15","doi-asserted-by":"crossref","first-page":"7623","DOI":"10.1016\/S0021-9258(19)81283-8","article-title":"Origin and possible significance of alanine production by skeletal muscle","volume":"249","author":"Odessey","year":"1974","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB16","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1111\/j.1365-2362.1982.tb02222.x","article-title":"Ammonia uptake by skeletal muscle in the hyperammonaemic rat","volume":"12","author":"Hod","year":"1982","journal-title":"Eur. J. Clin. Invest."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB17","doi-asserted-by":"crossref","first-page":"367","DOI":"10.1016\/0885-4505(86)90095-2","article-title":"Ammonia detoxification by accelerated oxidation of branched chain amino acids in brains of acute hepatic failure rats","volume":"35","author":"Watanabe","year":"1986","journal-title":"Biochem. Med. Metab. Biol."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB18","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1016\/S0168-8278(96)80249-2","article-title":"Hyperammonemia-induced depletion of glutamate and branched-chain amino acids in muscle and plasma","volume":"25","author":"Leweling","year":"1996","journal-title":"J. Hepatol."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB19","doi-asserted-by":"crossref","first-page":"3677","DOI":"10.1016\/S0021-9258(17)34854-8","article-title":"The origin of alanine produced in skeletal muscle","volume":"253","author":"Chang","year":"1978","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB20","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1172\/JCI108201","article-title":"Leucine. A possible regulator of protein turnover in muscle","volume":"56","author":"Buse","year":"1975","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB21","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1093\/jn\/130.2.139","article-title":"Orally administered leucine stimulates protein synthesis in skeletal muscle of postabsorptive rats in association with increased eIF4F formation","volume":"130","author":"Anthony","year":"2000","journal-title":"J. Nutr."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB22","doi-asserted-by":"crossref","first-page":"E466","DOI":"10.1152\/ajpendo.2001.281.3.E466","article-title":"Leucine and insulin activate p70 S6 kinase through different pathways in human skeletal muscle","volume":"281","author":"Greiwe","year":"2001","journal-title":"Am. J. Physiol. Endocrinol. Metab."},{"key":"10.1016\/j.ymgme.2003.11.017_BIB23","doi-asserted-by":"crossref","first-page":"2413","DOI":"10.1093\/jn\/130.10.2413","article-title":"Leucine stimulates translation initiation in skeletal muscle of postabsorptive rats via a rapamycin-sensitive pathway","volume":"130","author":"Anthony","year":"2000","journal-title":"J. Nutr."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719204000186?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719204000186?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,6,14]],"date-time":"2021-06-14T19:08:25Z","timestamp":1623697705000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719204000186"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,4]]},"references-count":23,"alternative-id":["S1096719204000186"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2003.11.017","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2004,4]]}}}