title: Lipoic acid improves survival in transgenic mouse models of Huntington's disease.
abstract: There is substantial evidence implicating excitotoxicity and oxidative damage in the pathogenesis of Huntington's disease (HD). We therefore examined whether the antioxidants 2-sulpho-tert-phenyibutyinitrone (S-PBN) and alpha-lipoic acid could exert neuroprotective effects in transgenic mouse models of HD. S-PBN showed no effects on either weight loss or survival in the R6/2 transgenic HD mice. alpha-Lipoic acid produced significant increases in survival in both R6/2 and N171-82Q transgenic mouse models of HD. These findings suggest that alpha-lipoic acid might have beneficial effects in HD patients.
authors.affiliation: Neurochemistry Laboratory, Neurology Service, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
authors.forename: O A
authors.initials: OA
authors.lastname: Andreassen
authors.forename: R J
authors.initials: RJ
authors.lastname: Ferrante
authors.forename: A
authors.initials: A
authors.lastname: Dedeoglu
authors.forename: M F
authors.initials: MF
authors.lastname: Beal
mesh_terms: D000818:Animals; D000975:Antioxidants / Q000494:pharmacology*; D001557:Benzenesulfonates / Q000494:pharmacology; D001835:Body Weight / Q000187:drug effects / Q000502:physiology; D001921:Brain / Q000187:drug effects* / Q000378:metabolism / Q000503:physiopathology; D004195:Disease Models, Animal; D005526:Food, Formulated; D006816:Huntington Disease / Q000188:drug therapy* / Q000378:metabolism / Q000503:physiopathology; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D018696:Neuroprotective Agents / Q000494:pharmacology*; D018384:Oxidative Stress / Q000187:drug effects* / Q000502:physiology; D013534:Survival / Q000502:physiology*; D008063:Thioctic Acid / Q000494:pharmacology*; D016896:Treatment Outcome
chemical_list: D000975:Antioxidants; D001557:Benzenesulfonates; D018696:Neuroprotective Agents; C094272:N-tert-butyl-(2-sulfophenyl)nitrone; D008063:Thioctic Acid