Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease

Ole A. Andreassen, Robert J. Ferrante, Hsueh Meei Huang, Alpaslan Dedeoglu, Larry Park, Kimberly L. Ferrante, Jennifer Kwon, David R. Borchelt, Christopher A Ross, Gary E. Gibson, M. Flint Beal

Research output: Contribution to journalArticle

Abstract

Dichloroacetate (DCA) stimulates pyruvate dehydrogenase complex (PDHC) activity and lowers cerebral lactate concentrations. In the R6/2 and N171-82Q transgenic mouse models of Huntington's disease (HD), DCA significantly increased survival, improved motor function, delayed loss of body weight, attenuated the development of striatal neuron atrophy, and prevented diabetes. The percentage of PDHC in the active form was significantly reduced in R6/2 mice at 12 weeks of age, and DCA ameliorated the deficit. These results provide further evidence for a role of energy dysfunction in HD pathogenesis and suggest that DCA may exert therapeutic benefits in HD.

Original languageEnglish (US)
Pages (from-to)112-117
Number of pages6
JournalAnnals of Neurology
Volume50
Issue number1
DOIs
StatePublished - 2001

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Huntington Disease
Therapeutic Uses
Transgenic Mice
Pyruvate Dehydrogenase Complex
Corpus Striatum
Atrophy
Lactic Acid
Body Weight
Neurons
Therapeutics

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Andreassen, O. A., Ferrante, R. J., Huang, H. M., Dedeoglu, A., Park, L., Ferrante, K. L., ... Beal, M. F. (2001). Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. Annals of Neurology, 50(1), 112-117. https://doi.org/10.1002/ana.1085

Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. / Andreassen, Ole A.; Ferrante, Robert J.; Huang, Hsueh Meei; Dedeoglu, Alpaslan; Park, Larry; Ferrante, Kimberly L.; Kwon, Jennifer; Borchelt, David R.; Ross, Christopher A; Gibson, Gary E.; Beal, M. Flint.

In: Annals of Neurology, Vol. 50, No. 1, 2001, p. 112-117.

Research output: Contribution to journalArticle

Andreassen, OA, Ferrante, RJ, Huang, HM, Dedeoglu, A, Park, L, Ferrante, KL, Kwon, J, Borchelt, DR, Ross, CA, Gibson, GE & Beal, MF 2001, 'Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease', Annals of Neurology, vol. 50, no. 1, pp. 112-117. https://doi.org/10.1002/ana.1085
Andreassen OA, Ferrante RJ, Huang HM, Dedeoglu A, Park L, Ferrante KL et al. Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. Annals of Neurology. 2001;50(1):112-117. https://doi.org/10.1002/ana.1085
Andreassen, Ole A. ; Ferrante, Robert J. ; Huang, Hsueh Meei ; Dedeoglu, Alpaslan ; Park, Larry ; Ferrante, Kimberly L. ; Kwon, Jennifer ; Borchelt, David R. ; Ross, Christopher A ; Gibson, Gary E. ; Beal, M. Flint. / Dichloroacetate exerts therapeutic effects in transgenic mouse models of Huntington's disease. In: Annals of Neurology. 2001 ; Vol. 50, No. 1. pp. 112-117.
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