Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease.
There is substantial evidence that excitotoxicity and oxidative damage may contribute to Huntington's disease (HD) pathogenesis. We examined whether the novel anti-oxidant compound BN82451 exerts neuroprotective effects in the R6/2 transgenic mouse model of HD. Oral administration of BN82451 significantly improved motor performance and improved survival by 15%. Oral administration of BN82451 significantly reduced gross brain atrophy, neuronal atrophy and the number of neuronal intranuclear inclusions at 90 days of age. These findings provide evidence that novel anti-oxidants such as BN82451 may be useful for treating HD.
Klivenyi
Peter
P
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York-Presbyterian Hospital, New York 10021, USA.
Ferrante
Robert J
RJ
Gardian
Gabrielle
G
Browne
Susan
S
Chabrier
Pierre-Etienne
PE
Beal
M Flint
MF
D000284:Administration, Oral; D000818:Animals; D000975:Antioxidants / Q000627:therapeutic use*; D001522:Behavior, Animal / Q000187:drug effects; D001921:Brain / Q000187:drug effects / Q000473:pathology; D004195:Disease Models, Animal; D006816:Huntington Disease / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D002479:Inclusion Bodies / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D051379:Mice; D008822:Mice, Transgenic; D009043:Motor Activity / Q000187:drug effects; D009474:Neurons / Q000187:drug effects / Q000378:metabolism / Q000473:pathology; D018696:Neuroprotective Agents / Q000627:therapeutic use*; D015996:Survival Rate; D016896:Treatment Outcome; D025801:Ubiquitin / Q000096:biosynthesis
D000975:Antioxidants; D018696:Neuroprotective Agents; D025801:Ubiquitin