{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,24]],"date-time":"2026-02-24T16:23:29Z","timestamp":1771950209980,"version":"3.50.1"},"reference-count":40,"publisher":"Elsevier BV","issue":"7","license":[{"start":{"date-parts":[[2017,7,1]],"date-time":"2017-07-01T00:00:00Z","timestamp":1498867200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2017,7,1]],"date-time":"2017-07-01T00:00:00Z","timestamp":1498867200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,2,3]],"date-time":"2023-02-03T00:00:00Z","timestamp":1675382400000},"content-version":"vor","delay-in-days":2043,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["The Journal of Nutrition"],"published-print":{"date-parts":[[2017,7]]},"DOI":"10.3945\/jn.117.248435","type":"journal-article","created":{"date-parts":[[2017,5,18]],"date-time":"2017-05-18T00:55:21Z","timestamp":1495068921000},"page":"1251-1257","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":13,"title":["Assessment of Nitric Oxide Production in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome with the Use of a Stable Isotope Tracer Infusion Technique"],"prefix":"10.1016","volume":"147","author":[{"given":"Ayman W","family":"El-Hattab","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Farook","family":"Jahoor","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.3945\/jn.117.248435_bib1","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.1126\/science.283.5407.1482","article-title":"Mitochondrial diseases in man and mouse","volume":"283","author":"Wallace","year":"1999","journal-title":"Science"},{"key":"10.3945\/jn.117.248435_bib2","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1016\/j.ceca.2016.03.003","article-title":"Mitochondrial cytopathies","volume":"60","author":"El-Hattab","year":"2016","journal-title":"Cell Calcium"},{"key":"10.3945\/jn.117.248435_bib3","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1002\/ana.410160409","article-title":"Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes: a distinctive clinical syndrome","volume":"16","author":"Pavlakis","year":"1984","journal-title":"Ann Neurol"},{"key":"10.3945\/jn.117.248435_bib4","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1038\/348651a0","article-title":"A mutation in the tRNA(Leu)(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies","volume":"348","author":"Goto","year":"1990","journal-title":"Nature"},{"key":"10.3945\/jn.117.248435_bib5","doi-asserted-by":"crossref","DOI":"10.1074\/jbc.M908734199","article-title":"The mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episode syndrome-associated human mitochondrial tRNALeu(UUR) mutation causes aminoacylation deficiency and concomitant reduced association of mRNA with ribosomes","volume":"275","author":"Chomyn","year":"2000","journal-title":"J Biol Chem"},{"key":"10.3945\/jn.117.248435_bib6","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1016\/j.ymgme.2015.06.004","article-title":"MELAS syndrome: clinical manifestations, pathogenesis, and treatment options","volume":"116","author":"El-Hattab","year":"2015","journal-title":"Mol Genet Metab"},{"key":"10.3945\/jn.117.248435_bib7","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1196\/annals.1444.011","article-title":"Mitochondrial encephalopathy, lactic acidosis, and strokelike episodes: basic concepts, clinical phenotype, and therapeutic management of MELAS syndrome","volume":"1142","author":"Sproule","year":"2008","journal-title":"Ann N Y Acad Sci"},{"key":"10.3945\/jn.117.248435_bib8","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.biocel.2013.12.009","article-title":"Mitochondria: role of citrulline and arginine supplementation in MELAS syndrome","volume":"48","author":"El-Hattab","year":"2014","journal-title":"Int J Biochem Cell Biol"},{"key":"10.3945\/jn.117.248435_bib9","doi-asserted-by":"crossref","first-page":"271","DOI":"10.1124\/pr.55.2.3","article-title":"The pharmacology of nitric oxide in the peripheral nervous system of blood vessels","volume":"55","author":"Toda","year":"2003","journal-title":"Pharmacol Rev"},{"key":"10.3945\/jn.117.248435_bib10","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/j.freeradbiomed.2010.04.004","article-title":"Subcellular and cellular locations of nitric oxide synthase isoforms as determinants of health and disease","volume":"49","author":"Villanueva","year":"2010","journal-title":"Free Radic Biol Med"},{"key":"10.3945\/jn.117.248435_bib11","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/bj3360001","article-title":"Arginine metabolism: nitric oxide and beyond","volume":"336","author":"Wu","year":"1998","journal-title":"Biochem J"},{"key":"10.3945\/jn.117.248435_bib12","doi-asserted-by":"crossref","DOI":"10.1093\/jn\/137.6.1602S","article-title":"Arginine metabolism: boundaries of our knowledge","volume":"137","author":"Morris","year":"2007","journal-title":"J Nutr"},{"key":"10.3945\/jn.117.248435_bib13","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1007\/s00726-005-0235-4","article-title":"Almost all about citrulline in mammals","volume":"29","author":"Curis","year":"2005","journal-title":"Amino Acids"},{"key":"10.3945\/jn.117.248435_bib14","doi-asserted-by":"crossref","first-page":"7749","DOI":"10.1073\/pnas.90.16.7749","article-title":"Plasma arginine and citrulline kinetics in adults given adequate and arginine-free diets","volume":"90","author":"Castillo","year":"1993","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.3945\/jn.117.248435_bib15","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1006\/bbrc.2000.3169","article-title":"Regulation of nitric oxide production by arginine metabolic enzymes","volume":"275","author":"Mori","year":"2000","journal-title":"Biochem Biophys Res Commun"},{"key":"10.3945\/jn.117.248435_bib16","doi-asserted-by":"crossref","first-page":"252","DOI":"10.1006\/niox.2001.0344","article-title":"Coinduction of endothelial nitric oxide synthase and arginine recycling enzymes in aorta of diabetic rats","volume":"5","author":"Oyadomari","year":"2001","journal-title":"Nitric Oxide"},{"key":"10.3945\/jn.117.248435_bib17","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1006\/niox.2001.0340","article-title":"Caveolar localization of arginine regeneration enzymes, argininosuccinate synthase, and lyase, with endothelial nitric oxide synthase","volume":"5","author":"Flam","year":"2001","journal-title":"Nitric Oxide"},{"key":"10.3945\/jn.117.248435_bib18","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1038\/nm.2544","article-title":"Requirement of argininosuccinate lyase for systemic nitric oxide production","volume":"17","author":"Erez","year":"2011","journal-title":"Nat Med"},{"key":"10.3945\/jn.117.248435_bib19","doi-asserted-by":"crossref","first-page":"710","DOI":"10.1212\/01.WNL.0000151976.60624.01","article-title":"L-arginine improves the symptoms of strokelike episodes in MELAS","volume":"64","author":"Koga","year":"2005","journal-title":"Neurology"},{"key":"10.3945\/jn.117.248435_bib20","doi-asserted-by":"crossref","first-page":"1766","DOI":"10.1212\/01.wnl.0000220197.36849.1e","article-title":"Endothelial dysfunction in MELAS improved by l-arginine supplementation","volume":"66","author":"Koga","year":"2006","journal-title":"Neurology"},{"key":"10.3945\/jn.117.248435_bib21","doi-asserted-by":"crossref","first-page":"2964","DOI":"10.1074\/mcp.M110.002964","article-title":"Increased protein nitration in mitochondrial diseases: evidence for vessel wall involvement","volume":"10","author":"Vattemi","year":"2011","journal-title":"Mol Cell Proteomics"},{"key":"10.3945\/jn.117.248435_bib22","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1016\/j.jns.2004.11.026","article-title":"Hypocitrullinemia in patients with MELAS: an insight into the \u201cMELAS paradox.\u201d","volume":"229\u2013230","author":"Naini","year":"2005","journal-title":"J Neurol Sci"},{"key":"10.3945\/jn.117.248435_bib23","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.mito.2006.11.006","article-title":"MELAS and L-arginine therapy","volume":"7","author":"Koga","year":"2007","journal-title":"Mitochondrion"},{"key":"10.3945\/jn.117.248435_bib24","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1016\/j.ymgme.2012.01.016","article-title":"Restoration of impaired nitric oxide production in MELAS syndrome with citrulline and arginine supplementation","volume":"105","author":"El-Hattab","year":"2012","journal-title":"Mol Genet Metab"},{"key":"10.3945\/jn.117.248435_bib25","doi-asserted-by":"crossref","first-page":"407","DOI":"10.1016\/j.ymgme.2016.01.010","article-title":"Impaired nitric oxide production in children with MELAS syndrome and the effect of arginine and citrulline supplementation","volume":"117","author":"El-Hattab","year":"2016","journal-title":"Mol Genet Metab"},{"key":"10.3945\/jn.117.248435_bib26","doi-asserted-by":"crossref","first-page":"771","DOI":"10.1016\/j.bbrc.2007.05.184","article-title":"The role of nitric oxide in muscle fibers with oxidative phosphorylation defects","volume":"359","author":"Tengan","year":"2007","journal-title":"Biochem Biophys Res Commun"},{"key":"10.3945\/jn.117.248435_bib27","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.tibtech.2004.12.001","article-title":"Metabolome analysis: the potential of in vivo labeling with stable isotopes for metabolite profiling","volume":"23","author":"Birkemeyer","year":"2005","journal-title":"Trends Biotechnol"},{"key":"10.3945\/jn.117.248435_bib28","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1006\/mben.2001.0187","article-title":"13C metabolic flux analysis","volume":"3","author":"Wiechert","year":"2001","journal-title":"Metab Eng"},{"key":"10.3945\/jn.117.248435_bib29","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1038\/nrg1880","article-title":"Inborn errors of metabolism: the flux from Mendelian to complex diseases","volume":"7","author":"Lanpher","year":"2006","journal-title":"Nat Rev Genet"},{"key":"10.3945\/jn.117.248435_bib30","first-page":"39","article-title":"Metabolic flux analysis using mass spectrometry","volume":"74","author":"Wittmann","year":"2002","journal-title":"Adv Biochem Eng Biotechnol"},{"key":"10.3945\/jn.117.248435_bib31","doi-asserted-by":"crossref","DOI":"10.1007\/BF02138782","article-title":"The investigation of inborn errors in vivo using stable isotopes","volume":"153","author":"Leonard","year":"1994","journal-title":"Eur J Pediatr"},{"key":"10.3945\/jn.117.248435_bib32","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1097\/00075197-200309000-00004","article-title":"Measuring in vivo metabolism using heavy water","volume":"6","author":"Dufner","year":"2003","journal-title":"Curr Opin Clin Nutr Metab Care"},{"key":"10.3945\/jn.117.248435_bib33","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1006\/mben.2001.0185","article-title":"Flux estimation using isotopic tracers: common ground for metabolic physiology and metabolic engineering","volume":"3","author":"Kelleher","year":"2001","journal-title":"Metab Eng"},{"key":"10.3945\/jn.117.248435_bib34","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1042\/CS20080444","article-title":"Arginine, citrulline and nitric oxide metabolism in sepsis","volume":"117","author":"Kao","year":"2009","journal-title":"Clin Sci (Lond)"},{"key":"10.3945\/jn.117.248435_bib35","doi-asserted-by":"crossref","first-page":"591","DOI":"10.1001\/jamaneurol.2015.5072","article-title":"Recommendations for the management of strokelike episodes in patients with mitochondrial encephalomyopathy, lactic acidosis, and strokelike episodes","volume":"73","author":"Koenig","year":"2016","journal-title":"JAMA Neurol"},{"key":"10.3945\/jn.117.248435_bib36","doi-asserted-by":"crossref","first-page":"443","DOI":"10.2165\/00023210-200620060-00002","article-title":"The mitochondrial myopathy encephalopathy, lactic acidosis with stroke-like episodes (MELAS) syndrome: a review of treatment options","volume":"20","author":"Scaglia","year":"2006","journal-title":"CNS Drugs"},{"key":"10.3945\/jn.117.248435_bib37","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/j.ymgme.2012.06.018","article-title":"Citrulline and arginine utility in treating nitric oxide deficiency in mitochondrial disorders","volume":"107","author":"El-Hattab","year":"2012","journal-title":"Mol Genet Metab"},{"key":"10.3945\/jn.117.248435_bib38","doi-asserted-by":"crossref","DOI":"10.1152\/ajpendo.00068.2010","article-title":"Arginine-induced stimulation of protein synthesis and survival in IPEC-J2 cells is mediated by mTOR but not nitric oxide","volume":"299","author":"Bauchart-Thevret","year":"2010","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"10.3945\/jn.117.248435_bib39","doi-asserted-by":"crossref","first-page":"2702","DOI":"10.1253\/circj.CJ-10-0044","article-title":"Abnormal myocardial energy-production state in mitochondrial cardiomyopathy and acute response to L-arginine infusion. C-11 acetate kinetics revealed by positron emission tomography","volume":"74","author":"Arakawa","year":"2010","journal-title":"Circ J"},{"key":"10.3945\/jn.117.248435_bib40","doi-asserted-by":"crossref","first-page":"615","DOI":"10.1016\/j.bbagen.2011.04.018","article-title":"In vivo functional brain imaging and a therapeutic trial of L-arginine in MELAS patients","volume":"1820","author":"Yoneda","year":"2012","journal-title":"Biochim Biophys Acta"}],"container-title":["The Journal of Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022316622162930?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022316622162930?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3945\/jn.117.248435","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,31]],"date-time":"2024-12-31T16:22:28Z","timestamp":1735662148000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0022316622162930"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,7]]},"references-count":40,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2017,7]]}},"alternative-id":["S0022316622162930"],"URL":"https:\/\/doi.org\/10.3945\/jn.117.248435","relation":{},"ISSN":["0022-3166"],"issn-type":[{"value":"0022-3166","type":"print"}],"subject":[],"published":{"date-parts":[[2017,7]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Assessment of Nitric Oxide Production in Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes Syndrome with the Use of a Stable Isotope Tracer Infusion Technique","name":"articletitle","label":"Article Title"},{"value":"The Journal of Nutrition","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.3945\/jn.117.248435","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2017 American Society for Nutrition. Published by Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.","name":"copyright","label":"Copyright"}]}}