title: Coenzyme Q10 and remacemide hydrochloride ameliorate motor deficits in a Huntington's disease transgenic mouse model.
abstract: 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.
authors.affiliation: Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA. schillin@mail.jhmi.edu
authors.forename: G
authors.initials: G
authors.lastname: Schilling
authors.forename: M L
authors.initials: ML
authors.lastname: Coonfield
authors.forename: C A
authors.initials: CA
authors.lastname: Ross
authors.forename: D R
authors.initials: DR
authors.lastname: Borchelt
mesh_terms: D000081:Acetamides / Q000627:therapeutic use*; D000818:Animals; D000975:Antioxidants / Q000627:therapeutic use*; D003067:Coenzymes; D004359:Drug Therapy, Combination; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology*; D051379:Mice; D008822:Mice, Transgenic / Q000235:genetics; D009043:Motor Activity / Q000187:drug effects*; D009419:Nerve Tissue Proteins / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D010446:Peptide Fragments / Q000235:genetics; D016019:Survival Analysis; D013997:Time Factors; D014451:Ubiquinone / Q000031:analogs & derivatives* / Q000627:therapeutic use*
chemical_list: D000081:Acetamides; D000975:Antioxidants; D003067:Coenzymes; C506812:Htt protein, mouse; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins; D010446:Peptide Fragments; D014451:Ubiquinone; C067553:remacemide; C024989:coenzyme Q10