DALLAS July 24, 2007 A drug used in some countries to treat the symptoms of Huntingtons disease prevents death of brain cells in mice genetically engineered to mimic the hereditary condition, UT Southwestern Medical Center researchers have found.

The research sheds light on the biochemical mechanisms involved in the disease and suggests new avenues of study for preventing brain-cell death in at-risk people before symptoms appear.

The drug can actually prevent brain cells from dying, said Dr. Ilya Bezprozvanny, associate professor of physiology at UT Southwestern. Its much more important than people thought.

The study, of which Dr. Bezprozvanny is senior author, appears in the July 25 issue of The Journal of Neuroscience.

The drug, called tetrabenazine (TBZ), is commercially distributed as Xenazine or Nitoman and blocks the action of dopamine, a compound that some nerve cells use to signal others. TBZ is approved for use in several countries, but not the U.S., to treat uncontrollable muscle movements in Huntingtons and other neurological conditions.

Huntingtons is a fatal genetic condition that usually manifests around ages 30 to 45, according to the Huntingtons Disease Society of America. About one in 10,000 people in America have the disease, with another 200,000 at risk. One of the most famous people with Huntingtons was folk singer Woody Guthrie, who died in 1967.

Huntingtons is caused by a dominant gene, meaning that a person carrying the gene is certain to develop the disease and has a 50 percent chance of passing it on to his or her children. Symptoms include jerky, uncontrollable movements called chorea and deterioration of reasoning abilities and personality. Symptoms begin after many brain cells have already died.

Although a genetic test exists, some people with a family history of Huntingtons choose not to be tested because there is no cure and because they fear loss of h
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Contact: Aline McKenzie
aline.mckenzie@utsouthwestern.edu
214-648-3404
UT Southwestern Medical Center
24-Jul-2007