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Protein Clue May Help Fight Huntington's Disease

Damage to brain cells may come from outside, too, scientists say

MONDAY, May 9, 2005 (HealthDay News) -- A mutant protein thought to be the culprit in Huntington's disease appears to trigger a form of cell toxicity that damages brain cells, according to new research.

The finding could lead to new therapeutic targets against the neurodegenerative disease, researchers say.

It's believed that Huntington's disease is caused by a mutant protein that develops in brain cells and kills them. In research with mice, a team at the University of California, Los Angeles, found evidence that this mutant protein also provokes toxic reactions from neighboring brain cells to generate HD, a fatal genetic disorder.

"This is really important because most current disease models and drug development efforts rely on the assumption that Huntington's disease arises from within the target brain cells," researcher Dr. William Yang, an assistant professor at the UCLA Neuropsyhiatric Institute and a member of the Brain Research Institute, said in a prepared statement.

"Our model is the first to show that mutant HD proteins exert their influence on brain cells located near the target cells. These neighboring cells then interact with the target cells to spark disease," Yang said.

The study appears in the May 5 issue of Neuron.

"Our next step will be determining how neighboring cells influence target cells and cause their death. Once we understand how these cells interact, the knowledge may lead to new therapeutic strategies to treat Huntington's disease," he said.

More information

The U.S. National Institute of Neurological Disorders and Stroke has more about Huntington's disease.

SOURCE: University of California, Los Angeles, news release, May 4, 2005
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