{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,24]],"date-time":"2026-03-24T19:49:57Z","timestamp":1774381797269,"version":"3.50.1"},"reference-count":48,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2021,10,28]],"date-time":"2021-10-28T00:00:00Z","timestamp":1635379200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002322","name":"Coordena\u00e7\u00e3o de Aperfei\u00e7oamento de Pessoal de N\u00edvel Superior","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002322","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2022,4]]},"abstract":"Abstract<\/jats:title>\n \n Glutaric acidemia type I (GA\u2010I) is an inborn error of metabolism of lysine, hydroxylysine, and tryptophan, caused by glutaryl\u2010CoA\u2010dehydrogenase (GCDH) deficiency, characterized by the buildup of toxic organic acids predominantly in the brain. After acute catabolic states, patients usually develop striatal degeneration, but the mechanisms behind this damage are still unknown. Quinolinic acid (QA), a metabolite of the kynurenine pathway, increases especially during infections\/inflammatory processes, and could act synergically with organic acids, contributing to the neurological features of GA\u2010I. The aim of this study was to investigate whether QA increases seizure susceptibility and modifies brain oscillation patterns in an animal model of GA\u2010I, the\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n mice taking high\u2010lysine diet (\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys). Therefore, the characteristics of QA\u2010induced seizures and changes in brain oscillatory patterns were evaluated by video\u2010electroencephalography (EEG) analysis recorded in\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n \u2010\n <\/jats:italic>\n Lys,\n \n Gcdh\n +\/+<\/jats:sup>\n \u2010\n <\/jats:italic>\n Lys, and\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010N (normal diet) animals. We found that the number of seizures\n per<\/jats:italic>\n animal was similar for all groups receiving QA,\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys\u2010QA,\n Gcdh<\/jats:italic>\n +\/+<\/jats:sup>\n \u2010Lys\u2010QA, and\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010N\u2010QA. However, severe seizures were observed in the majority of\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys\u2010QA mice (82%), and only in 25% of\n Gcdh<\/jats:italic>\n +\/+<\/jats:sup>\n \u2010Lys\u2010QA and 44% of\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010N\u2010QA mice. All\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys animals developed spontaneous recurrent seizures (SRS), but\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys\u2010QA animals had increased number of SRS, higher mortality rate, and significant predominance of lower frequency oscillations on EEG. Our results suggest that QA plays an important role in the neurological features of GA\u2010I, as\n \n Gcdh\n \u2212\/\u2212<\/jats:sup>\n <\/jats:italic>\n \u2010Lys mice exhibit increased susceptibility to intrastriatal QA\u2010induced seizures and long\u2010term changes in brain oscillations.\n <\/jats:p>","DOI":"10.1002\/jnr.24980","type":"journal-article","created":{"date-parts":[[2021,10,28]],"date-time":"2021-10-28T20:55:28Z","timestamp":1635454528000},"page":"992-1007","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Increased susceptibility to quinolinic acid\u2010induced seizures and long\u2010term changes in brain oscillations in an animal model of glutaric acidemia type I"],"prefix":"10.1002","volume":"100","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5698-8693","authenticated-orcid":false,"given":"Let\u00edcia","family":"Barbieri Caus","sequence":"first","affiliation":[{"name":"Neurophysiology and 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