Huntington's Disease Research - Genetics, Causes, Symptoms, Treatment

Huntington's Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Huntington's Disease, including details on genetics, causes, symptoms, treatment.


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Ethyl-EPA treatment improves motor dysfunction, but not neurodegeneration in the YAC128 mouse model of Huntington disease.

Van Raamsdonk JM, Pearson J, Rogers DA, Lu G, Barakauskas VE, Barr AM, Honer WG, Hayden MR, Leavitt BR

Department of Medical Genetics and Centre for Molecular Medicine and Therapeutics, British Columbia Research Institute for Children's and Women's Health, University of British Columbia, 980 West 28th Avenue, Vancouver, BC, Canada V5Z 4H4.

Huntington disease (HD) is an adult-onset neurodegenerative disorder that is characterized by selective degeneration in the striatum. There are currently no treatments that can prevent the progressive decline of motor and cognitive function in HD. In parallel with a human clinical trial, we examined the efficacy of ethyl-EPA treatment in the YAC128 mouse model of HD. Oral delivery of ethyl-EPA to symptomatic YAC128 mice beginning at 7 months of age increased membrane EPA levels 3-fold (P < 0.001) and resulted in a modest but significant improvement in motor dysfunction by 12 months of age as measured by open-field activity (P = 0.01) and performance on the rotarod (P = 0.05). At this age, ethyl-EPA-treated YAC128 mice showed no improvement in striatal volume, striatal neuron counts, striatal neuronal cross-sectional area, or striatal DARPP-32 expression compared to untreated YAC128 mice, thereby indicating no reduction of striatal neuropathology. This result is congruent with modest motor benefits observed in HD patients treated with ethyl-EPA. Overall, this work demonstrates the feasibility of experimental therapeutics in the YAC128 mouse model and suggests that experiments in these mice may be predictive for future human clinical trials.

Published 18 November 2005 in Exp Neurol, 196(2): 266-72.
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Huntington's Disease Research Today Archive:

Volume 1 (2005)
  Issue 1 (January)
  Issue 2 (February)
  Issue 3 (March)
  Issue 4 (April)
  Issue 5 (May)
  Issue 6 (June)
  Issue 7 (July)
  Issue 8 (August)
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  Issue 12 (December)

Volume 2 (2006)
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  Issue 3 (March)
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Volume 3 (2007)
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  Issue 10 (October)
  Issue 11 (November)
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Volume 4 (2008)
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  Issue 7 (July)
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  Issue 10 (October)



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