Mouse study holds promise for humans with hereditary disease
THURSDAY, Dec. 24 (HealthDay News) -- Researchers are reporting that they've discovered a molecular switch that appears to play a role in whether Huntington's disease develops in mice.
An estimated one out of every 10,000 Americans suffer from Huntington's disease, which is hereditary and disrupts a person's ability to walk, talk, think clearly and even swallow. There's currently no way to prevent the disease, which typically leads to death within 10 years of diagnosis, or to keep it from worsening, the study authors noted.
Scientists have found that a mutation in a protein called huntingtin causes the disease to develop. But there's still plenty of mystery about how that happens.
"It's unclear how the mutant protein causes age-related and progressive loss of brain cells in patients with Huntington's disease," X. William Yang, an associate professor of psychiatry and biobehavioral sciences at the Semel Institute of Neuroscience and Human Behavior at University of California Los Angeles, said in a news release from the school. He's senior author of a study on the finding that appears in the Dec. 24 issue of the journal Neuron.
In the study, researchers reported that they've gained new insight into how Huntington's develops by examining the process in mice.
"Our study identified a critical molecular switch...," Yang said. "This finding suggests an exciting new avenue to develop therapeutics for Huntington's disease."
The National Institute of Neurological Disorders and Stroke has more information on Huntington's disease.
-- Randy Dotinga
SOURCE: University of California Los Angeles, news release, Dec. 24, 2009
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