{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:39:40Z","timestamp":1787315980841,"version":"build-2736575974"},"reference-count":37,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,1,25]],"date-time":"2014-01-25T00:00:00Z","timestamp":1390608000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Great Ormond Street Hospital Children's Charity"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Neurochemistry"],"published-print":{"date-parts":[[2014,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    The Ketogenic diet (\n                    <jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>\n                    ) is an effective treatment with regards to treating pharmaco\u2010resistant epilepsy. However, there are difficulties around compliance and tolerability. Consequently, there is a need for refined\/simpler formulations that could replicate the efficacy of the\n                    <jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>\n                    . One of the proposed hypotheses is that the\n                    <jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>\n                    increases cellular mitochondrial content which results in elevation of the seizure threshold. Here, we have focussed on the medium\u2010chain triglyceride form of the diet and the observation that plasma octanoic acid (C8) and decanoic acid (C10) levels are elevated in patients on the medium\u2010chain triglyceride\n                    <jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>\n                    . Using a neuronal cell line (\n                    <jats:styled-content style=\"fixed-case\">SH<\/jats:styled-content>\n                    \u2010\n                    <jats:styled-content style=\"fixed-case\">SY<\/jats:styled-content>\n                    5Y), we demonstrated that 250\u2010\u03bcM C10, but not C8, caused, over a 6\u2010day period, a marked increase in the mitochondrial enzyme, citrate synthase along with complex I activity and catalase activity. Increased mitochondrial number was also indicated by electron microscopy. C10 is a reported peroxisome proliferator activator receptor \u03b3 agonist, and the use of a peroxisome proliferator activator receptor \u03b3 antagonist was shown to prevent the C10\u2010mediated increase in mitochondrial content and catalase. C10 may mimic the mitochondrial proliferation associated with the\n                    <jats:styled-content style=\"fixed-case\">KD<\/jats:styled-content>\n                    and raises the possibility that formulations based on this fatty acid could replace a more complex diet.\n                    <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                      <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc12646-fig-0004-m.png\">\n                        <jats:alt-text>image<\/jats:alt-text>\n                      <\/jats:graphic>\n                    <\/jats:boxed-text>\n                  <\/jats:p>\n                  <jats:p>We propose that decanoic acid (C10) results in increased mitochondrial number. Our data suggest that this may occur via the activation of the PPAR\u03b3 receptor and its target genes involved in mitochondrial biogenesis. This finding could be of significant benefit to epilepsy patients who are currently on a strict ketogenic diet. Evidence that C10 on its own can modulate mitochondrial number raises the possibility that a simplified and less stringent C10\u2010based diet could be developed.<\/jats:p>","DOI":"10.1111\/jnc.12646","type":"journal-article","created":{"date-parts":[[2014,1,3]],"date-time":"2014-01-03T00:34:49Z","timestamp":1388709289000},"page":"426-433","source":"Crossref","is-referenced-by-count":181,"title":["The ketogenic diet component decanoic acid increases mitochondrial citrate synthase and complex I activity in neuronal cells"],"prefix":"10.1111","volume":"129","author":[{"given":"Sean David","family":"Hughes","sequence":"first","affiliation":[{"name":"Clinical and Molecular Genetics Unit UCL Institute of Child Health London UK"},{"name":"Chemical Pathology Great Ormond Street Hospital for Children NHS Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marta","family":"Kanabus","sequence":"additional","affiliation":[{"name":"Clinical and Molecular Genetics Unit UCL Institute of Child Health London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Glenn","family":"Anderson","sequence":"additional","affiliation":[{"name":"Electron Microscopy Unit Great Ormond Street Hospital, Foundation Trust London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iain P.","family":"Hargreaves","sequence":"additional","affiliation":[{"name":"Neurometabolic Unit National Hospital for Neurology and Neurosurgery London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tricia","family":"Rutherford","sequence":"additional","affiliation":[{"name":"Vitaflo International Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maura O\u2019","family":"Donnell","sequence":"additional","affiliation":[{"name":"Vitaflo International Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. 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