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Neurological disorder impacts brain cells differently
Date:11/9/2011

Bergmann glia (support cells found in the cerebellum) and cells in the olivary complex (part of the brainstem controlling body movement).

By creating and comparing mice that expressed the mutant gene only in targeted cells, La Spada said the scientists made two unexpected discoveries: First, when the gene mutation was eliminated from Bergmann glia, neurodegeneration continued unabated and still involved dysfunction and degeneration of the Bergmann glia themselves. Second, when the mutation was excised from Purkinje neurons and the olivary complex, there was significantly less neurological damage and Bergmann glia remained intact.

"The first result highlights the relatively new idea that degeneration goes both ways," said La Spada. "It isn't just neurons becoming affected when their support cells dysfunction. The Bergmann glia didn't express the mutant gene, but they still degenerated. This shows the bilateral relationship between neurons and non-neuronal cells. They're equal partners, in both normal functioning and in disease.

"The second result underscores the relevance of Purkinje cells and the olivary neuron circuit in the brainstem to SCA7. When it's dysfunctional, degeneration occurs. This is crucial for our understanding of this disease, and should enable us to develop more specific therapeutic approaches. Although we have our work cut out for us, we now have a better idea of what we're up against."


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Contact: Scott LaFee
slafee@ucsd.edu
619-543-6163
University of California - San Diego
Source:Eurekalert  

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