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Mouse Results Suggest Possible Drug Treatment for Huntington’s Disease

Researchers have found that normal synaptic activity in nerve cells (the electrical activity in the brain that allows nerve cells to communicate with one another) protects the brain from the misfolded proteins associated with Huntington's disease. In contrast, excessive extrasynaptic activity (aberrant electrical activity in the brain, usually not associated with communication between nerve cells) enhances the misfolded proteins' deadly effects. In addition, the scientists found that the drug Memantine, which is approved to treat Alzheimer's disease, successfully treated Huntington's disease in a mouse model by preserving normal synaptic electrical activity and suppressing excessive extrasynaptic electrical activity. "Chronic neurodegenerative diseases like Huntington's, Alzheimer's, and Parkinson's are all related to protein misfolding," said Dr. Stuart Lipton of the Burnham Institute for Medical Research, senior author of the report. "We show here, for the first time, that electrical activity controls protein folding, and if you have a drug that can adjust the electrical activity to the correct levels, you can protect against misfolding. Also, this verifies that appropriate electrical activity is protective, supporting the 'use it or lose it theory' of brain activity at the molecular level. For example, this finding may explain why epidemiologists have found that 'using' your brain by performing crossword puzzles and other games can stave off cognitive decline in diseases like Alzheimer's." A small human clinical trial of Memantine for Huntington's disease has recently shown positive effects. Larger, international clinical trials are now being planned. In addition to Dr. Lipton, the article’s authors included Dr. Michael Hayden of the University of British Columbia. The article was published online on November 15 in Nature Medicine. [Press release] [Nature Medicine abstract]