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Lipoic acid improves survival in transgenic mouse models of Huntington's disease

Andreassen, Ole A.; Ferrante, Robert J.1; Dedeoglu, Alpaslan1; Beal, M. Flint2, CA

Clinical Neuroscience

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 α-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. α-Lipoic acid produced significant increases in survival in both R6/2 and N171-82Q transgenic mouse models of HD. These findings suggest that α-lipoic acid might have beneficial effects in HD patients.

Author Information

Neurochemistry Laboratory, Neurology Service, Massachusetts General Hospital and Harvard Medical School, Boston, MA; 1Geriatric Research Education and Clinical Center, Bedford VA Medical Center, Bedford, MA and Boston University School of Medicine, Boston, MA; 2Department of Neurology and Neuroscience, Weill Medical College of Cornell University, New York Hospital-Cornell Medical Center, 525 East 68th Street, New York, NY 10021, USA

CACorresponding Author

Received 17 July 2001; accepted 15 August 2001

© 2001 Lippincott Williams & Wilkins, Inc.