Triheptanoin does not improve physical performance in people with Tarui disease

In a double-blind, placebo-controlled crossover trial, a Danish team evaluated the effects of 14 days of tripheptanoin in three people with phosphofructokinase deficiency (PFKD) or type VII glycogenosis (Tarui disease) on exercise heart rate and fatty acid oxidation.

The idea was to short-circuit the deficit in energy production by the Krebs cycle resulting from the blockage of pyruvate production, by using the beta-oxidation of triheptanoin to provide succinyl-CoA which could then enter the Krebs cycle.

  • Treatment with triheptanoin for 14 days does not lower heart rate or improve fatty acid oxidation during exercise.
  • Triheptanoin increased plasma palmitate production in all three participants during exercise.
  • There was no difference in glucose production, utilisation and oxidation under triheptanoin and placebo.
  • Triheptanoin did not affect perception of exercise, maximal O2 consumption or maximal work in a progressive exercise test.
  • Participants did not feel better on triheptanoin.


No effect of triheptanoin in patients with phosphofructokinase deficiency. Raaschou-Pedersen DE, Madsen KL, Løkken N, Storgaard JH, Quinlivan R, Laforêt P, Lund A, Van Hall G, Vissing J, Ørngreen M. Neuromuscul Disord. 2022 Feb 4:S0960-8966(22)00029-3.