Lipoic acid improves survival in transgenic mouse models of Huntington's disease.
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.
Andreassen
O A
OA
Neurochemistry Laboratory, Neurology Service, Massachusetts General Hospital and Harvard Medical School, Boston, MA, USA.
Ferrante
R J
RJ
Dedeoglu
A
A
Beal
M F
MF
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
D000975:Antioxidants; D001557:Benzenesulfonates; D018696:Neuroprotective Agents; C094272:N-tert-butyl-(2-sulfophenyl)nitrone; D008063:Thioctic Acid