Increased survival and neuroprotective effects of BN82451 in a transgenic mouse model of Huntington's disease.

Abstract

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.

• Authors: Peter Klivenyi, Robert J Ferrante, Gabrielle Gardian, Susan Browne, Pierre-Etienne Chabrier, M Flint Beal
• Journal: Journal of neurochemistry (J Neurochem) Vol. 86 Issue 1 Pg. 267-72 (Jul 2003) ISSN: 0022-3042 [Print] England
• PMID: 12807446 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Antioxidants
• Neuroprotective Agents
• Ubiquitin

Topics

• Administration, Oral
• Animals
• Antioxidants (therapeutic use)
• Behavior, Animal (drug effects)
• Brain (drug effects, pathology)
• Disease Models, Animal
• Huntington Disease (drug therapy, genetics, pathology)
• Inclusion Bodies (drug effects, metabolism, pathology)
• Mice
• Mice, Transgenic
• Motor Activity (drug effects)
• Neurons (drug effects, metabolism, pathology)
• Neuroprotective Agents (therapeutic use)
• Survival Rate
• Treatment Outcome
• Ubiquitin (biosynthesis)