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Tetrabenazine (TBZ) Protects Brain Cells in Huntington's Disease Model

Filed in archive Studies , Treatment by Gloria Gamat on July 26, 2007

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The drug called tetrabenazine (TBZ), used in some countries for the treatment of the symptomslinks of Huntington's disease has been found to prevent the death of brain cells in mice models, according to UT Southwestern Medical Center researchers.

Huntington's 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.


TBZ -commercially distributed as Xenazine™ (Cambridge Laboratories) or Nitoman® (Roche) - blocks the action of dopamine (a compound that some nerve cells use to signal others). Though not approved in the United States, TBZ is approved for use in several countries to treat uncontrollable muscle movements in Huntington's and other neurological conditions.

The UT Soutwestern findings shed light on the biochemical mechanisms involved in Huntington's disease and suggests new avenues of study for preventing brain-cell death in at-risk people before symptoms appear.

Although there is already a genetic test for Huntington's disease, some people with a family history opt not to be tested because there is no cure and because they fear loss of health insurance.

All treatments available only lessen the symptoms, but there is currently no way to slow or halt the progression of the Huntington's disease.

Find more details from the full report.


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