{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T13:23:19Z","timestamp":1784208199520,"version":"3.55.0"},"reference-count":60,"publisher":"American Society for Clinical Investigation","issue":"22","license":[{"start":{"date-parts":[[2025,11,24]],"date-time":"2025-11-24T00:00:00Z","timestamp":1763942400000},"content-version":"vor","delay-in-days":39,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","award":["5R01HD10360"],"award-info":[{"award-number":["5R01HD10360"]}]},{"name":"national institute of health","award":["DK090242"],"award-info":[{"award-number":["DK090242"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,11,24]]},"abstract":"<jats:p>\n                    Long-chain fatty acid oxidation disorders (LC-FAODs) cause energy deficits in heart and skeletal muscle that are only partially corrected by current medium-chain lipid therapies such as triheptanoin. We find that heart and muscle lack medium-chain acyl-CoA synthetases, limiting the capacity for \u03b2-oxidation of medium-chain fatty acids. Instead, heart and muscle mitochondria robustly respire on medium-chain acylcarnitines. The mitochondrial matrix enzyme carnitine acetyltransferase (CrAT) efficiently converts orally delivered octanoylcarnitine (C\n                    <jats:sub>8<\/jats:sub>\n                    -carnitine) to octanoyl-CoA for energy generation. C\n                    <jats:sub>8<\/jats:sub>\n                    -carnitine exhibits twice the oral bioavailability of triheptanoin and distributes to muscle and heart. A single oral dose significantly enhances grip strength and treadmill endurance while attenuating lactic acidosis in 2 mouse models of LC-FAODs. Thus, medium-chain acylcarnitines overcome a previously unrecognized metabolic bottleneck in LC-FAOD muscle and may represent an alternative to triglyceride-based therapies for bioenergetic disorders.\n                  <\/jats:p>","DOI":"10.1172\/jci.insight.199443","type":"journal-article","created":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T16:00:22Z","timestamp":1760630422000},"source":"Crossref","is-referenced-by-count":6,"title":["Oral octanoylcarnitine alleviates exercise intolerance in mouse models of long-chain fatty acid oxidation disorders"],"prefix":"10.1172","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4212-4832","authenticated-orcid":true,"given":"Keaton J.","family":"Solo","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Division of Genetic and Genomic Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, 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