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Huntington's Disease Linked to High Brain Cholesterol

Progressive neurological illness may stem from lipid overdelivery

FRIDAY, Dec. 8, 2006 (HealthDay News) -- U.S. researchers have spotted a link between Huntington's disease and abnormal levels of cholesterol in the brain.

Huntington's disease is a progressive brain condition in which the brain's nerve cells waste away. Symptoms include uncontrolled movements, emotional problems, and mental deterioration.

In tests with cultured neuron cells in the laboratory and in the brains of animals, a team at the Mayo Clinic in Rochester, Minn., found that the mutated Huntington's protein interacts with another protein to disrupt the normal cholesterol-delivery system, resulting in the accumulation of large amounts of cholesterol in the brain.

The findings are published in the current issue of the journal Human Molecular Genetics.

"Cholesterol is essential for promoting the connection network among brain cells and in maintaining their membrane integrity. But the level of cholesterol and its delivery to the proper locations in the cell are essential for the survival of neurons," molecular biologist Cynthia McMurray explained in a prepared statement.

"Our discovery that the mutant Huntington's disease protein derails the cholesterol delivery and causes cholesterol accumulation in neurons provides us with key results and solid clues to the mechanism of this disease. Fully understanding the mechanism of toxicity is the key to developing treatments," she said.

About 30,000 Americans have Huntington's disease, and another 150,000 carry the Huntington's gene and have a 50 percent risk of passing it on to their children.

More information

We Move has more about Huntington's disease.

SOURCE: Mayo Clinic, news release, Dec. 1, 2006
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