{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T17:16:17Z","timestamp":1768410977433,"version":"3.49.0"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2020,8,4]],"date-time":"2020-08-04T00:00:00Z","timestamp":1596499200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Anaplerotic odd\u2010chain fatty acid supplementation has been suggested as an approach to replenish citric acid cycle intermediate (CACi) pools and facilitate adenosine triphosphate (ATP) production in subjects with long\u2010chain fatty acid oxidation disorders, but the evidence that cellular CACi depletion exists and that repletion occurs following anaplerotic substrate supplementation is limited. We exercised very long\u2010chain acyl\u2010CoA dehydrogenase\u2010deficient (VLCAD\u2212\/\u2212) and wild\u2010type (WT) mice to exhaustion and collected cardiac tissue for measurement of CACi by targeted metabolomics. In a second experimental group, VLCAD\u2212\/\u2212 and WT mice that had been fed chow prepared with either medium\u2010chain triglyceride (MCT) oil or triheptanoin for 4\u2009weeks were exercised for 60\u2009minutes. VLCAD\u2212\/\u2212 mice exhibited lower succinate in cardiac muscle at exhaustion than WT mice suggesting lower CACi in VLCAD\u2212\/\u2212 with prolonged exercise. In mice fed either MCT or triheptanoin, succinate and malate were greater in VLCAD\u2212\/\u2212 mice fed triheptanoin compared to VLCAD\u2212\/\u2212 animals fed MCT but lower than WT mice fed triheptanoin. Long\u2010chain odd acylcarnitines such as C19 were elevated in VLCAD\u2212\/\u2212 and WT mice fed triheptanoin suggesting some elongation of the heptanoate, but it is unknown what proportion of heptanoate was oxidized vs elongated. Prolonged exercise was associated with decreased cardiac muscle succinate in VLCAD\u2212\/\u2212 mice in comparison to WT mice. VLCAD\u2212\/\u2212 fed triheptanoin had increased succinate compared to VLCAD\u2212\/\u2212 mice fed MCT but lower than WT mice fed triheptanoin. Cardiac CACi were higher following dietary ingestion of an anaplerotic substrate, triheptanoin, in comparison to MCT.<\/jats:p>","DOI":"10.1002\/jimd.12284","type":"journal-article","created":{"date-parts":[[2020,7,10]],"date-time":"2020-07-10T11:44:02Z","timestamp":1594381442000},"page":"1232-1242","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Cardiac tissue citric acid cycle intermediates in exercised very long\u2010chain <scp>acyl\u2010CoA<\/scp> dehydrogenase\u2010deficient mice fed triheptanoin or medium\u2010chain triglyceride"],"prefix":"10.1002","volume":"43","author":[{"given":"Garen","family":"Gaston","sequence":"first","affiliation":[{"name":"Department of Molecular and Medical Genetics Oregon Health &amp; Science University  Portland Oregon USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jon A.","family":"Gangoiti","sequence":"additional","affiliation":[{"name":"Department of 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