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1 Pediatrics, Case Western Reserve University, Cleveland, Ohio, United States; Nutrition, Case Western Reserve University, Cleveland, Ohio, United States
2 Nutrition, Case Western Reserve University, Cleveland, Ohio, United States
3 Fachhochschule Lippe und Hoxter University of Applied Sciences, Detmold, Germany
4 N/A, SASOL GmbH, Witten, Germany
5 Institute of Metabolic Disease, Baylor University Medical Center, Dallas, Texas, United States
* To whom correspondence should be addressed. E-mail: hxb8{at}case.edu.
A new chronic treatment for inherited disorders of long-chain fatty acid oxidation involves administering up to one-third of dietary calories as triheptanoin, a medium-odd-chain triglyceride (Roe et al J. Clin. Invest. 110: 259, 2002). Heptanoate and C5-ketone bodies derived from its partial oxidation in liver are precursors of anaplerotic propionyl-CoA in peripheral tissues. It was hypothesized that increasing anaplerosis in peripheral tissues would boost energy production. In the present study, we tested the potential of a triheptanoin emulsion as an intravenous nutrient. Normal rats were infused with triheptanoin intravenously or intraduodenally at up to 40% of caloric requirement. The blood concentration ratio (heptanoate)/(C5-ketone bodies) was high with intravenous, and low with intraduodenal triheptanoin infusion. During intravenous infusion of triheptanoin, lipolysis was stimulated, but appeared compensated by fatty acid re-esterification. During intraduodenal infusion of triheptanoin, lipolysis was not stimulated. Our data support the hypothesis that intravenous triheptanoin could be used to treat decompensated patients with long-chain fatty acid oxidation disorders.
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