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Mouse model confirms mutated protein's role in dementia
Date:11/2/2010

A team of scientists from Japan and the University of California, San Diego School of Medicine have created a new mouse model that confirms that mutations of a protein called beta-synuclein promote neurodegeneration. The discovery creates a potential new target for developing treatments of diseases like Parkinson's and Alzheimer's.

The work is published in today's issue of Nature Communications. Lead author is Makoto Hashimoto of the Division of Chemistry and Metabolism, Tokyo Metropolitan Institute for Neuroscience, with colleagues including Eliezer Masliah, MD, professor of neurosciences and pathology in the UC San Diego School of Medicine, Edward Rockenstein, a research associate in UCSD's Experimental Neuropath Laboratory and Albert R. La Spada, MD, PhD, professor of cellular and molecular medicine, chief of the Division of Genetics in the Department of Pediatrics and associate director of the Institute for Genomic Medicine at UC San Diego.

In 2004, La Spada discovered mutations in a family afflicted with a neurological disorder known as Dementia with Lewy Bodies. DLB is one of the most common types of progressive dementia, combining features of both Alzheimer's and Parkinson's diseases. Lewy bodies are abnormal aggregates of proteins. There are no known therapies to stop or slow the DLB's progression. There is no cure.

In the 2004 study, La Spada and colleagues found that mutations of the naturally occurring B-synuclein protein in DLB patients "were strong strongly suggestive of being pathogenic." That is, the mutated protein caused or was a cause of the disease. But the findings were not definitive.

The newly published research describes the creation of a transgenic mouse model that expresses the B-synuclein mutation. The mice suffer from neurodegenerative disease, validating La Spada's earlier work.

"Beta-synuclein is interesting because it is closely related to alpha-synuclein, a protein that can c
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Contact: Scott LaFee
slafee@ucsd.edu
619-543-6163
University of California - San Diego
Source:Eurekalert  

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Mouse model confirms mutated protein's role in dementia
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