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Closing in on Rett syndrome

Study shows that location in the brain matters when it comes to the syndrome

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3:24pm, September 24, 2008
A study released in the Sept. 25 Neuron is a major step toward identifying the brain regions behind the behaviors that characterize Rett syndrome, a debilitating, autism-like neurological disease that primarily affects females.

The syndrome is marked by a constellation of symptoms, the most striking of which is repetitive hand wringing. Behavioral symptoms of the syndrome include a lack of language skills, muscle rigidity that imparts a characteristic tremor, high anxiety and, in some cases, excessive aggression.

Rett syndrome is caused by a damaged copy of a gene called MeCP2, which is located on the X chromosome. Because the gene is expressed throughout the brain, finding the discrete regions that control the long list of individual behavioral symptoms associated with the syndrome has proven exceptionally hard.

For help, scientists have turned to mice. Animals lacking the protein MeCP2 in all brain regions behave simila

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