Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease

@article{Mochel2010DietaryAT,
  title={Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease},
  author={Fanny Mochel and Sandrine Duteil and C Marelli and C{\'e}line Jauffret and Agn{\`e}s Barles and Janette Holm and Lawrence Sweetman and J. P. Benoist and Daniel Rabier and Pierre G. Carlier and Alexandra Durr},
  journal={European Journal of Human Genetics},
  year={2010},
  volume={18},
  pages={1057-1060}
}
We previously identified a systemic metabolic defect associated with early weight loss in patients with Huntington's disease (HD), suggesting a lack of substrates for the Krebs cycle. Dietary anaplerotic therapy with triheptanoin is used in clinical trials to promote energy production in patients with peripheral and brain Krebs cycle deficit, as its metabolites – C5 ketone bodies – cross the blood–brain barrier. We conducted a short-term clinical trial in six HD patients (UHDRS (Unified… CONTINUE READING

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