Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model

Neurosci Lett. 2001 Nov 27;315(3):149-53. doi: 10.1016/s0304-3940(01)02326-6.


Huntington's disease (HD) is a progressive inherited neurodegenerative disorder, for which there is no effective therapy. The CARE-HD study, recently published, evaluated the ability of a combination of coenzyme Q10 (CoQ10) and remacemide hydrochloride (R) to ameliorate symptoms, which might arise from glutamate-mediated excitotoxicity and abnormalities in mitochondrial energy production. In this study, we examined the efficacy of CoQ10/R therapy on ameliorating the motor dysfunction and premature death of HD-N171-82Q transgenic mice. Motor performance, measured on the Rotarod, was specifically but transiently improved beginning 3 weeks after initiating the CoQ10/R therapy. Survival, however was not prolonged. Our findings suggest that further study of CoQ10/R in mouse models is warranted to investigate whether this therapeutic approach can ameliorate the symptoms of HD in early stages of the disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acetamides / therapeutic use*
  • Animals
  • Antioxidants / therapeutic use*
  • Coenzymes
  • Drug Therapy, Combination
  • Huntingtin Protein
  • Huntington Disease / drug therapy*
  • Huntington Disease / mortality
  • Huntington Disease / physiopathology*
  • Mice
  • Mice, Transgenic / genetics
  • Motor Activity / drug effects*
  • Nerve Tissue Proteins / genetics
  • Nuclear Proteins / genetics
  • Peptide Fragments / genetics
  • Survival Analysis
  • Time Factors
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / therapeutic use*


  • Acetamides
  • Antioxidants
  • Coenzymes
  • Htt protein, mouse
  • Huntingtin Protein
  • Nerve Tissue Proteins
  • Nuclear Proteins
  • Peptide Fragments
  • Ubiquinone
  • remacemide
  • coenzyme Q10