Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease

J Neurosci. 2002 Mar 1;22(5):1592-9. doi: 10.1523/JNEUROSCI.22-05-01592.2002.


There is substantial evidence that bioenergetic defects and excitotoxicity may play a role in the pathogenesis of Huntington's disease (HD). Potential therapeutic strategies for neurodegenerative diseases in which there is reduced energy metabolism and NMDA-mediated excitotoxicity are the administration of the mitochondrial cofactor coenzyme Q10 and the NMDA antagonist remacemide. We found that oral administration of either coenzyme Q10 or remacemide significantly extended survival and delayed the development of motor deficits, weight loss, cerebral atrophy, and neuronal intranuclear inclusions in the R6/2 transgenic mouse model of HD. The combined treatment, using coenzyme Q10 and remacemide together, was more efficacious than either compound alone, resulting in an approximately 32 and 17% increase in survival in the R6/2 and N171-82Q mice, respectively. Magnetic resonance imaging showed that combined treatment significantly attenuated ventricular enlargement in vivo. These studies further implicate defective energy metabolism and excitotoxicity in the R6/2 and N171-82Q transgenic mouse models of HD and are of interest in comparison with the outcome of a recent clinical trial examining coenzyme Q10 and remacemide in HD patients.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, Non-P.H.S.
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Acetamides / therapeutic use*
  • Administration, Oral
  • Animals
  • Behavior, Animal / drug effects
  • Body Weight / drug effects
  • Brain / drug effects
  • Brain / pathology
  • Cerebral Ventricles / drug effects
  • Cerebral Ventricles / pathology
  • Coenzymes
  • Disease Models, Animal
  • Disease Progression
  • Drug Evaluation, Preclinical
  • Drug Synergism
  • Female
  • Humans
  • Huntingtin Protein
  • Huntington Disease / drug therapy*
  • Huntington Disease / genetics
  • Huntington Disease / pathology
  • Magnetic Resonance Imaging
  • Male
  • Mice
  • Mice, Transgenic
  • Motor Activity / drug effects
  • Nerve Tissue Proteins / genetics
  • Nuclear Proteins / genetics
  • Organ Size / drug effects
  • Survival Rate
  • Treatment Outcome
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / therapeutic use*


  • Acetamides
  • Coenzymes
  • HTT protein, human
  • Htt protein, mouse
  • Huntingtin Protein
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • Ubiquinone
  • remacemide
  • coenzyme Q10