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In some cases, the exogenous CoQ supplementation has limited efficacy. In the\n                    <jats:italic>Coq9<\/jats:italic>\n                    <jats:sup>\n                      <jats:italic>R239X<\/jats:italic>\n                    <\/jats:sup>\n                    mouse model with fatal mitochondrial encephalopathy due to CoQ deficiency, we have tested the therapeutic potential of \u03b2\u2010resorcylic acid (\u03b2\u2010RA), a structural analog of the CoQ precursor 4\u2010hydroxybenzoic acid and the anti\u2010inflammatory salicylic acid. \u03b2\u2010RA noticeably rescued the phenotypic, morphological, and histopathological signs of the encephalopathy, leading to a significant increase in the survival. Those effects were due to the decrease of the levels of demethoxyubiquinone\u20109 (DMQ\n                    <jats:sub>9<\/jats:sub>\n                    ) and the increase of mitochondrial bioenergetics in peripheral tissues. However, neither CoQ biosynthesis nor mitochondrial function changed in the brain after the therapy, suggesting that some endocrine interactions may induce the reduction of the astrogliosis, spongiosis, and the secondary down\u2010regulation of astrocytes\u2010related neuroinflammatory genes. Because the therapeutic outcomes of \u03b2\u2010RA administration were superior to those after CoQ\n                    <jats:sub>10<\/jats:sub>\n                    supplementation, its use in the clinic should be considered in CoQ deficiencies.\n                  <\/jats:p>","DOI":"10.15252\/emmm.201809466","type":"journal-article","created":{"date-parts":[[2018,11,27]],"date-time":"2018-11-27T10:45:25Z","timestamp":1543315525000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["\u03b2\u2010RA reduces DMQ\/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice"],"prefix":"10.1038","volume":"11","author":[{"given":"Agust\u00edn","family":"Hidalgo\u2010Guti\u00e9rrez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eliana","family":"Barriocanal\u2010Casado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed","family":"Bakkali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M Elena","family":"D\u00edaz\u2010Casado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"S\u00e1nchez\u2010Maldonado","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miguel","family":"Romero","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ramy K","family":"Sayed","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cornelia","family":"Prehn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Germaine","family":"Escames","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Duarte","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dar\u00edo","family":"Acu\u00f1a\u2010Castroviejo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3355-0298","authenticated-orcid":false,"given":"Luis C","family":"L\u00f3pez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2018,11,11]]},"reference":[{"key":"BFEMMM201809466_CR1","doi-asserted-by":"crossref","first-page":"1241","DOI":"10.1016\/j.fct.2004.12.018","volume":"43","author":"TB Adams","year":"2005","unstructured":"Adams TB, Cohen SM, Doull J, Feron VJ, Goodman JI, Marnett LJ, Munro IC, Portoghese PS, Smith RL, Waddell WJ et\u00a0al (2005) The FEMA GRAS assessment of hydroxy\u2010 and alkoxy\u2010substituted benzyl derivatives used as flavor ingredients. 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