{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,20]],"date-time":"2026-09-20T07:57:52Z","timestamp":1789891072428,"version":"4.0.1"},"reference-count":32,"publisher":"Public Library of Science (PLoS)","issue":"2","license":[{"start":{"date-parts":[[2015,2,3]],"date-time":"2015-02-03T00:00:00Z","timestamp":1422921600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/publicdomain\/zero\/1.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0116594","type":"journal-article","created":{"date-parts":[[2015,2,3]],"date-time":"2015-02-03T18:57:39Z","timestamp":1422989859000},"page":"e0116594","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":10,"title":["A New Mouse Model of Mild Ornithine Transcarbamylase Deficiency (spf-j) Displays Cerebral Amino Acid Perturbations at Baseline and upon Systemic Immune Activation"],"prefix":"10.1371","volume":"10","author":[{"given":"Tatyana N.","family":"Tarasenko","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Odrick R.","family":"Rosas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Larry N.","family":"Singh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kara","family":"Kristaponis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hilary","family":"Vernon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter J.","family":"McGuire","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2015,2,3]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1042\/bj1760733","article-title":"Sources of ammonia for mammalian urea synthesis","volume":"176","author":"HA Krebs","year":"1978","journal-title":"Biochem J"},{"key":"ref2","first-page":"263","article-title":"Disorders of the urea cycle and related enzymes","author":"J Fernandes","year":"2006"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"S29","DOI":"10.1007\/s00431-003-1347-z","article-title":"Long-term outcome of patients with urea cycle disorders and the question of neonatal screening","volume":"162","author":"C Bachmann","year":"2003","journal-title":"Eur J Pediatr"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1500","DOI":"10.1056\/NEJM198406073102304","article-title":"Neurologic outcome in children with inborn errors of urea synthesis. Outcome of urea-cycle enzymopathies","volume":"310","author":"M Msall","year":"1984","journal-title":"N Engl J Med"},{"key":"ref5","doi-asserted-by":"crossref","unstructured":"Krivitzky L, Babikian T, Lee H, Hattiangadi Thomas N, Burk-Paull KL, et al. (2009) Intellectual, Adaptive, and Behavioral Functioning in Children with Urea Cycle Disorders. Pediatr Res.","DOI":"10.1203\/PDR.0b013e3181a27a16"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"482","DOI":"10.1056\/NEJM198002283020902","article-title":"Cerebral dysfunction in asymptomatic carriers of ornithine transcarbamylase deficiency","volume":"302","author":"ML Batshaw","year":"1980","journal-title":"N Engl J Med"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1007\/s11011-012-9348-0","article-title":"Urea cycle defects and hyperammonemia: effects on functional imaging","volume":"28","author":"AL Gropman","year":"2013","journal-title":"Metab Brain Dis"},{"key":"ref8","doi-asserted-by":"crossref","unstructured":"Pacheco-Colon I, Fricke S, VanMeter J, Gropman AL (2014) Advances in urea cycle neuroimaging: Proceedings from the 4th International Symposium on urea cycle disorders, Barcelona, Spain, September 2013. Mol Genet Metab.","DOI":"10.1016\/j.ymgme.2014.05.005"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1016\/j.ymgme.2010.01.017","article-title":"Brain imaging in urea cycle disorders","volume":"100","author":"A Gropman","year":"2010","journal-title":"Mol Genet Metab"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1007\/s10545-007-0718-4","article-title":"Nutritional management of patients with urea cycle disorders","volume":"30","author":"RH Singh","year":"2007","journal-title":"J Inherit Metab Dis"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"S6","DOI":"10.1067\/mpd.2001.111831","article-title":"Proceedings of a consensus conference for the management of patients with urea cycle disorders","volume":"138","author":"M Summar","year":"2001","journal-title":"J Pediatr"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1093\/hmg\/6.6.963","article-title":"\u2018Late onset\u2019 ornithine transcarbamylase deficiency: function of three purified recombinant mutant enzymes","volume":"6","author":"H Morizono","year":"1997","journal-title":"Human molecular genetics"},{"issue":"Pt 2","key":"ref13","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1042\/bj3220625","article-title":"Expression, purification and kinetic characterization of wild-type human ornithine transcarbamylase and a recurrent mutant that produces \u2018late onset\u2019 hyperammonaemia","volume":"322","author":"H Morizono","year":"1997","journal-title":"The Biochemical journal"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1002\/1096-8628(20000814)93:4<313::AID-AJMG11>3.0.CO;2-M","article-title":"Genotype spectrum of ornithine transcarbamylase deficiency: Correlation with the clinical and biochemical phenotype","volume":"93","author":"BA McCullough","year":"2000","journal-title":"American Journal of Medical Genetics"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1002\/jssc.200900758","article-title":"Orotic acid quantification in dried blood spots and biological fluids by hydrophilic interaction liquid chromatography tandem mass spectrometry","volume":"33","author":"O D\u2019Apolito","year":"2010","journal-title":"J Sep Sci"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1016\/0003-2697(81)90594-7","article-title":"An Improved Colorimetric Assay for Aspartate and Ornithine Transcarbamylases","volume":"118","author":"SC Pastralandis","year":"1981","journal-title":"Analytical Biochemistry"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"e2345","DOI":"10.1371\/journal.pone.0002345","article-title":"Functional diversification of paralogous transcription factors via divergence in DNA binding site motif and in expression","volume":"3","author":"LN Singh","year":"2008","journal-title":"PLoS One"},{"key":"ref18","first-page":"1030","article-title":"Remutations at The Jackson Laboratory (Update to Mouse Genome 1993; 91:862\u20135 - J16313)","volume":"93","author":"HO Sweet","year":"1995","journal-title":"Mouse Genome"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"727","DOI":"10.1258\/0004563001899807","article-title":"Severe hyperammonaemia in a previously healthy teenager","volume":"37","author":"PJ Galloway","year":"2000","journal-title":"Annals of clinical biochemistry"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1016\/j.ymthe.2006.03.009","article-title":"Long-term correction of ammonia metabolism and prolonged survival in ornithine transcarbamylase-deficient mice following liver-directed treatment with adeno-associated viral vectors","volume":"14","author":"D Moscioni","year":"2006","journal-title":"Molecular therapy: the journal of the American Society of Gene Therapy"},{"key":"ref21","first-page":"94","article-title":"Animal Models of Otc Deficiency and Their Gene Therapy","author":"PJ Snodgrass","year":"2004"},{"key":"ref22","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":"NE Maestri","year":"1992","journal-title":"The Journal of pediatrics"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1016\/j.jhep.2004.08.021","article-title":"Involvement of natural killer cells in PolyI:C-induced liver injury","volume":"41","author":"Z Dong","year":"2004","journal-title":"J Hepatol"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1136\/jmg.33.8.645","article-title":"Phenotypic variability in male patients carrying the mutant ornithine transcarbamylase (OTC) allele, Arg40His, ranging from a child with an unfavourable prognosis to an asymptomatic older adult","volume":"33","author":"I Matsuda","year":"1996","journal-title":"Journal of medical genetics"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1136\/jmg.33.8.645","article-title":"Phenotypic variability in male patients carrying the mutant ornithine transcarbamylase (OTC) allele, Arg40His, ranging from a child with an unfavourable prognosis to an asymptomatic older adult","volume":"33","author":"I Matsuda","year":"1996","journal-title":"J Med Genet"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/j.jhep.2009.11.014","article-title":"Adult-onset ornithine transcarbamylase (OTC) deficiency unmasked by the Atkins\u2019 diet","volume":"52","author":"Z Ben-Ari","year":"2010","journal-title":"Journal of hepatology"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1016\/j.nut.2013.01.022","article-title":"Branched-chain amino acids and ammonia metabolism in liver disease: therapeutic implications","volume":"29","author":"M Holecek","year":"2013","journal-title":"Nutrition"},{"key":"ref28","doi-asserted-by":"crossref","DOI":"10.1016\/j.jpeds.2013.08.029","article-title":"Infectious precipitants of acute hyperammonemia are associated with indicators of increased morbidity in patients with urea cycle disorders","volume":"163","author":"PJ McGuire","year":"2013","journal-title":"The Journal of pediatrics"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1002\/glia.20027","article-title":"Role of glial amino acid transporters in synaptic transmission and brain energetics","volume":"47","author":"P Marcaggi","year":"2004","journal-title":"Glia"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/j.bbrc.2003.07.012","article-title":"Amino acid signalling and the integration of metabolism","volume":"313","author":"AJ Meijer","year":"2004","journal-title":"Biochem Biophys Res Commun"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"2068S","DOI":"10.1093\/jn\/133.6.2068S","article-title":"Interorgan amino acid transport and its regulation","volume":"133","author":"JT Brosnan","year":"2003","journal-title":"J Nutr"},{"key":"ref32","article-title":"Investigating neurological deficits in carriers and affected patients with ornithine transcarbamylase deficiency","author":"C Sprouse","year":"2014","journal-title":"Mol Genet Metab"}],"container-title":["PLOS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0116594","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,8,20]],"date-time":"2019-08-20T15:34:47Z","timestamp":1566315287000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0116594"}},"subtitle":[],"editor":[{"given":"Fran\u00e7ois","family":"Blachier","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2015,2,3]]},"references-count":32,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2015,2,3]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0116594","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2015,2,3]]}}}