Using a mouse model, reserchers now understand changes occurring in the brain before the onset of Huntington's disease

New highly efficient DNA array technology brings researchers one step closer to understanding the progression of Huntington's disease and the potential for the development of therapies

Huntington's disease affects nearly 30,000 people nationwide, with another 150,000 at risk for inheriting the disease. Like a genetic time bomb, one abnormal gene in Huntington's victims disrupts the brain's nerve cell functions, slowly degrading physical, intellectual and emotional capability and leading inevitably to death.

In a collaborative study conducted by the Fred Hutchinson Cancer Research Center (FHCRC) in Seattle and Massachusetts General Hospital (MGH) in Boston, researchers used a mouse model to identify changes that occur in nerve cells in the early stages of Huntington's disease. They identified several signaling molecules that could be targeted for future therapies. The results are reported in the May 22 issue of the journal Human Molecular Genetics.

Equally important to the knowledge gained is that the research team was able to do in six months what would have taken years in the past. Using new technologies in FHCRC's DNA Array Lab the team used genetic microchips--called microarrays--to survey the patterns of active and non-active genes at early and late symptomatic stages in a mouse model of Huntington's disease.

The research teams, led by Drs. Jim Olson, a pediatric oncologist at FHCRC, University of Washington and Children's Hospital and Regional Medical Center, and Ruth Luthi-Carter, a neurobiologist at MGH, monitored nearly 6,000 genes in the brain region most damaged by Huntington's disease. They found a small subset of genes to be affected by the disease, only two percent of the total. Importantly, many of the affected genes code the molecules that brain cells need to receive and process messages.

"The mouse models provide a window into the early stages of the disease," says Luthi-Carter. "This unders

Contact: Susan Edmonds
Fred Hutchinson Cancer Research Center

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