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Patients with MSUD present a series of neurological dysfunction. Recent studies have been associated the brain damage in the MSUD with inflammation and immune system activation. MSUD patients die within a few months of life due to recurrent metabolic crises and neurologic deterioration, often precipitated by infection or other stresses. In this regard, our previous results showed that the inflammatory process, induced by lipopolysaccharide (LPS), associated with high levels of BCAAs causes blood\u2010brain barrier (BBB) breakdown due to hyperactivation of MMPs. Thus, we hypothesize that the synergistic action between high concentrations of BCAAs (H\u2010BCAAs) and LPS on BBB permeability and hyperactivation of MMPs could be through an increase in the production of cytokines and RAGE protein levels. We observed that high levels of BCAA in infant rats are related to increased brain inflammation induced by LPS administration. In addition, BCAA exposure led to an increase on brain RAGE expression of young rats. The brain inflammation was characterized by enhanced levels of interleukin 1 \u03b2 (IL\u20101\u03b2), interleukin 6 (IL\u20106), tumor necrosis factor\u2010\u03b1 (TNF\u2010\u03b1) and Interferon\u2010 \u03b3 (IFN\u2010\u03b3), and decreased content of interleukin\u201010 (IL\u201010). Therefore, MSUD is associated with a more intense neuroinflammation induced by LPS infection.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2019.07.007","type":"journal-article","created":{"date-parts":[[2019,7,19]],"date-time":"2019-07-19T15:34:41Z","timestamp":1563550481000},"page":"210-214","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Administration of branched\u2010chain amino acids increases the susceptibility to lipopolysaccharide\u2010induced inflammation in young Wistar rats"],"prefix":"10.1002","volume":"78","author":[{"given":"Leticia B.","family":"Wessler","sequence":"first","affiliation":[{"name":"Laborat\u00f3rio de Neurologia Experimental, Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Crici\u00fama SC Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vitor","family":"de Miranda Ramos","sequence":"additional","affiliation":[{"name":"Centro de Estudos em Estresse Oxidativo, Departamento de Bioqu\u00edmica, ICBS Universidade Federal do Rio Grande do Sul Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matheus Augusto","family":"Bittencourt Pasquali","sequence":"additional","affiliation":[{"name":"Centro de Estudos em Estresse Oxidativo, Departamento de Bioqu\u00edmica, ICBS Universidade Federal do Rio Grande do Sul Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 Cl\u00e1udio","family":"Fonseca Moreira","sequence":"additional","affiliation":[{"name":"Centro de Estudos em Estresse Oxidativo, Departamento de Bioqu\u00edmica, ICBS Universidade Federal do Rio Grande do Sul Porto Alegre RS Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jade","family":"de Oliveira","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Neurologia Experimental, Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Crici\u00fama SC Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giselli","family":"Scaini","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Neurologia Experimental, Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Crici\u00fama SC Brazil"},{"name":"Translational Psychiatry Program Department of Psychiatry and Behavioral Sciences McGovern Medical School The University of Texas Health Science Center at Houston (UTHealth) Houston TX USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emilio L.","family":"Streck","sequence":"additional","affiliation":[{"name":"Laborat\u00f3rio de Neurologia Experimental, Programa de P\u00f3s\u2010Gradua\u00e7\u00e3o em Ci\u00eancias da Sa\u00fade Universidade do Extremo Sul Catarinense Crici\u00fama SC Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2019,7,19]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2004.09.008"},{"key":"e_1_2_6_3_1","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1093\/jn\/109.7.1214","article-title":"Prevention of the immunodepressive effects of excess dietary leucine by isoleucine and valine in the rat","volume":"109","author":"Aschkenasy A.","year":"1979","journal-title":"J. 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